{"title":"Reducing radiation exposure in pediatric cervical spine imaging for trauma: a multi-disciplinary quality improvement initiative.","authors":"Nina Yu, Jonathan Emerson Kohler, Kendra Grether-Jones, Maureen Murphy, Marike Zwienenberg","doi":"10.1007/s00381-025-06754-z","DOIUrl":"10.1007/s00381-025-06754-z","url":null,"abstract":"<p><strong>Purpose: </strong>Pediatric cervical spine injury (PCSI) can result in devastating neurologic disability. While computed tomography (CT) imaging is both sensitive and specific in detecting clinically significant injuries, indiscriminate utilization can lead to excessive ionizing radiation exposure. A routine institutional audit revealed CTs were inappropriately obtained 54% of the time. This study evaluates the effects of an updated protocol to reduce radiation exposure in pediatric trauma patients.</p><p><strong>Methods: </strong>Data were retrospectively analyzed from a pediatric level 1 trauma center from 2021 to 2022. The data were divided into two cohorts, pre-implementation (2021) and post-implementation (2022). Inclusion criteria were patients 0-14 years old with a Glasgow Coma Scale (GCS) ranging 9-15. Outside-hospital transfers were excluded. The primary study endpoints were guideline compliance and CT utilization.</p><p><strong>Results: </strong>A total of 82 subjects were enrolled in this study. In 2021, there were 38 subjects (female/male 15/23, mean age 5.9 years old) with an average GCS of 13.6. In 2022, there were 44 subjects (female/male 19/25, mean age 5.2 years old) with an average GCS of 14.0. In 2021, the overall protocol adherence rate was 81.6%, and post-implementation in 2022, compliance was 93.2% (p = 0.109). Following implementation, the rate of inappropriate (protocol non-adherent CT) use decreased from 58.6 to 6.8% (p < 0.05).</p><p><strong>Conclusions: </strong>Implementation of a new evidence-based institutional protocol for PCSI was associated with improved adherence and reduction of unnecessary CT orders. Ongoing monitoring will help determine if these improvements are sustained.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":"41 1","pages":"102"},"PeriodicalIF":1.3,"publicationDate":"2025-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11790755/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143122045","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}
Eeshan Khurana, Justice Welch, John Collins, Adam Ammar, Catherine A Mazzola
{"title":"Surgical complication index for pediatric patients (SCIPP): A novel pediatric frailty index predicting postoperative complications in a study of 133 pediatric neurosurgical patients.","authors":"Eeshan Khurana, Justice Welch, John Collins, Adam Ammar, Catherine A Mazzola","doi":"10.1007/s00381-025-06752-1","DOIUrl":"https://doi.org/10.1007/s00381-025-06752-1","url":null,"abstract":"<p><strong>Purpose: </strong>A predictive index for surgical complications in pediatric patients is lacking in the current scientific literature. We have developed a simple index to accurately predict the likelihood of complications after surgery. The Surgical Complication Index for Pediatric Patients (SCIPP) is reliable and accurate for both heterogenous and specific groups of pediatric patients.</p><p><strong>Methods: </strong>A retrospective analysis of pediatric neurosurgical procedures from 2005 to 2023 was conducted. Logistic and linear regression analyses were performed to analyze the correlation with their respective SCIPP scores and post-operative complications and length of stay (LOS).</p><p><strong>Results: </strong>Our cohort included 90 tethered cord patients and 43 surgeries consisting of craniotomies/craniectomies, laminectomies/laminotomies, and others. The mean SCIPP score across all 133 patients was 2.41 ± 1.76, with an average age of 5.37 ± 5.53 years. From the logistic regression, each 1-point increase in SCIPP score was associated with increased odds of experiencing a complication after surgery in all patients (odds ratio: 1.57; p < 0.001) and tethered cord patients (odds ratio: 1.59; p = 0.007). The reduced 4-point SCIPP was associated with increased odds in all patients (odds ratio: 2.67; p < 0.001) and tethered cord patients (odds ratio: 2.89; p = 0.001) as well. Upon linear regression analysis, each 1-point increase in SCIPP was associated with a 0.49 day increase in LOS (p = 0.002). Each 1-point increase in reduced SCIPP was associated with a 0.42 day increase in LOS but was not statistically significant (p = 0.120).</p><p><strong>Conclusion: </strong>The SCIPP is a simple and accurate tool that predicts surgical complications and LOS in a variety of pediatric neurosurgical procedures and can be used to counsel patients and families on the risks of surgery.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":"41 1","pages":"101"},"PeriodicalIF":1.3,"publicationDate":"2025-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143078725","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}
Emily Xu, Emily Zhang, Kristen Park, Mahaa Ayub, Chao Zhao, Jimmy W Huh, J Michael King, Iris Paltin, Amish C Shah, Phillip B Storm, Alexander Tucker, Peter J Madsen, Shih-Shan Lang
{"title":"Medical management of cerebellar mutism syndrome at a quaternary children's hospital.","authors":"Emily Xu, Emily Zhang, Kristen Park, Mahaa Ayub, Chao Zhao, Jimmy W Huh, J Michael King, Iris Paltin, Amish C Shah, Phillip B Storm, Alexander Tucker, Peter J Madsen, Shih-Shan Lang","doi":"10.1007/s00381-025-06759-8","DOIUrl":"10.1007/s00381-025-06759-8","url":null,"abstract":"<p><strong>Purpose: </strong>We aimed to evaluate the efficacy of selective serotonin reuptake inhibitors (SSRIs) in treating cerebellar mutism syndrome (CMS).</p><p><strong>Methods: </strong>We retrospectively reviewed all pediatric patients who underwent a posterior fossa tumor resection between May 2007 to September 2022 at a single quaternary pediatric hospital. We evaluated clinical presentation and hospital course, including imaging findings, pathology, and surgical approaches. Propensity score matching was used to compare the symptom duration of patients who received SSRIs versus those who did not.</p><p><strong>Results: </strong>A total of 292 patients met the criteria with 25% (n = 73) being diagnosed with CMS. Several factors were significantly associated with a CMS diagnosis, such as pre-operative hydrocephalus (p = 0.002), a vermis-splitting approach (p = 0.007), tumor in the fourth ventricle (p = 0.010), medulloblastoma diagnosis (p = 0.009), and postoperative complication (p < 0.001). Of the patients diagnosed with CMS, 32.9% (n = 24) received SSRI treatment, specifically fluoxetine (n = 18) and sertraline (n = 6). Overall, treatment did not decrease the duration of CMS symptoms or shorten the inpatient rehab course compared to matched controls. However, within the cohort of fluoxetine-treated patients, earlier initiation of medication was significantly correlated with a shorter duration of mutism (p = 0.007).</p><p><strong>Conclusions: </strong>We report the largest cohort of CMS patients treated with SSRIs. The lack of overall clinical benefit when compared to untreated patients in our study may be due to the length of delay in starting an SSRI, since early initiation of fluoxetine correlated with shorter CMS symptoms. These results support the importance of early clinical detection of CMS and potentially treating CMS early in the patient's postoperative course.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":"41 1","pages":"100"},"PeriodicalIF":1.3,"publicationDate":"2025-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11790773/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143078652","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":"Spinal epidural lipomatosis in pediatric medulloblastoma following chemotherapy, a case report and review of the literature.","authors":"Mandisa L Keswa, Hung N Tran, Darian R Esfahani","doi":"10.1007/s00381-025-06757-w","DOIUrl":"https://doi.org/10.1007/s00381-025-06757-w","url":null,"abstract":"<p><strong>Purpose: </strong>Spinal epidural lipomatosis (SEL) is a relatively rare condition characterized by overgrowth of adipose tissue in the extradural space of the spinal canal. Compression of neural structures can lead to radiculopathy, loss of sensation and motor function, and urinary retention. Known causes of SEL include exogenous steroid use and obesity.</p><p><strong>Methods: </strong>We describe the unique case of idiopathic SEL in a 1-year-old male, the youngest reported to date, diagnosed with medulloblastoma and treated with chemotherapy and autologous stem cell rescue (AuSCR). We then perform a systemic review of the literature of this rare diagnosis, explore potential causes, and review management.</p><p><strong>Results: </strong>The patient was non-obese and received a cumulative dose of only 60 mg dexamethasone (150 mg/m<sup>2</sup>), far below pediatric cancer patients with SEL documented in the literature. The acute presentation of lipomatosis following chemotherapy and AuSCR, associated with transient elevated triglycerides (234 mg/dL), suggests this as a possible cause.</p><p><strong>Conclusion: </strong>This case illustrates the development and resolution of SEL in a non-obese pediatric cancer patient with limited steroid use. While further study is needed to identify the etiology of SEL, this diagnosis should be evaluated for when reviewing spine imaging following chemotherapy or autologous stem cell rescue.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":"41 1","pages":"99"},"PeriodicalIF":1.3,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143074056","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":"Prospective analysis on possible changes of cognitive functions in children on follow-up for brain tumor.","authors":"Ilaria Liguoro, Chiara Pilotto, Francesco Tuniz, Maristella Toniutti, Paola Cogo, Tiziana Zilli","doi":"10.1007/s00381-025-06751-2","DOIUrl":"https://doi.org/10.1007/s00381-025-06751-2","url":null,"abstract":"<p><strong>Purpose: </strong>This study was aimed at evaluating cognitive functioning in children with brain tumors before and after surgery and at the end of all adjunctive treatments.</p><p><strong>Methods: </strong>All newly diagnosed children with primary brain tumor were prospectively assessed. Neurocognitive evaluations were performed before surgery (T0), within 7-10 days from surgery (T1) and 18-24 months after the end of all treatments (T2). Language, memory, attention, executive functions, visual-constructional, and sensorimotor skills were evaluated at T0, T1, and T2, whereas intelligent quotient (IQ) was explored at T0 and T2.</p><p><strong>Results: </strong>Twenty-five patients (M:F = 15:10, mean age 10.9 ± 3.4 years) were enrolled between January 2019 and December 2022. At baseline, patients showed major deficits in narrative memory (6.6 ± 3.7, p < 0.001) and visuo-spatial memory (copy design tasks: general 6.8 ± 3.9, p < 0.001; specific 6.2 ± 3.3, p < 0.001; and motor 5.2 ± 3.2, p < 0.001). In the post-surgery phase (T1), significant deficits remained in narrative memory (6.2 ± 3.3, p < 0.001) and visual-motor coordination (copy design tasks: specific 5.9 ± 3.0, p < 0.001; motor 4.8 ± 4.3 p < 0.001), while attention and visual-constructional abilities significantly improved (p = 0.04 and p = 0.001 respectively). Nine out of 25 patients (36%) reached the T2 evaluation: persistency of deficits in the area of visuo-spatial processing and a possible decline in median IQ values in comparison to T0 evaluation (93 vs 100, p = 0.05) were shown.</p><p><strong>Conclusions: </strong>Children with brain tumors may present several neuropsychological impairments since diagnosis. Surgery may have a positive impact in relation to the recovery of some cognitive functions. However, cognitive decline may worsen over time. Baseline and periodic neurocognitive evaluations should be encouraged to indicate targets for cognitive monitoring, to early detect functional difficulties.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":"41 1","pages":"97"},"PeriodicalIF":1.3,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143074032","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":"Reflexive symbolism: The extensor cutaneous plantar reflex in Cabanel's The birth of Venus.","authors":"Francesco Brigo","doi":"10.1007/s00381-025-06758-9","DOIUrl":"https://doi.org/10.1007/s00381-025-06758-9","url":null,"abstract":"","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":"41 1","pages":"98"},"PeriodicalIF":1.3,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143074053","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}
Mayur S Patel, Kathleen S Botterbush, Tyler N Lackland, Michael Prim, Noor Al-Hammadi, Matthew Shorey, Tobias A Mattei, Philippe A Mercier
{"title":"Intraoperative neuromonitoring is not a useful adjunct for Chiari malformation decompressive surgery: a cost-benefit and legal analysis.","authors":"Mayur S Patel, Kathleen S Botterbush, Tyler N Lackland, Michael Prim, Noor Al-Hammadi, Matthew Shorey, Tobias A Mattei, Philippe A Mercier","doi":"10.1007/s00381-024-06738-5","DOIUrl":"https://doi.org/10.1007/s00381-024-06738-5","url":null,"abstract":"<p><strong>Objectives: </strong>Posterior fossa decompression is currently an operative treatment of choice for Chiari Malformation I (CM1). However, there is controversy surrounding the possible benefits of employing intraoperative neuromonitoring (INM) for this type of procedure. In addition to presenting our single-center experience on the use of INM, we analyze the cost associated with INM in Chiari Malformation (CM) decompression surgery using the Healthcare Cost and Utilization Project (HCUP) database and discuss the legal implications of somatosensory evoked potentials (SSEP) monitoring during decompression for CM1.</p><p><strong>Methods: </strong>We conducted a retrospective review of all patients undergoing CM1 decompression with SSEP neuromonitoring from 2011 to 2018. We collected patient characteristics, hospital charges, and surgical cost data from the HCUP database for patients undergoing CM decompression. Finally, we performed a review within the Thompson Reuters Westlaw Edge database for reported litigation involving INM for CM decompression.</p><p><strong>Results: </strong>None of the 110 patients submitted to surgery for CM1 at our institution had any significant SSEP changes intraoperatively or developed post-operative neurological deterioration. There were higher mean total hospital charges and surgical costs associated with INM ($31,272) for patients who received INM compared to patients who did not receive INM ($24,112). A careful review of the Westlaw database with multiple-word search strategies revealed no reported medical malpractice claims regarding the absence of SSEP neuromonitoring in a CM decompression procedure.</p><p><strong>Conclusion: </strong>Using data collected at our institution and the HCUP national database, we showed that intraoperative neuromonitoring did not affect surgical planning and decision-making or post operative care, while adding unnecessary costs to CM decompression procedures. The absence of reported malpractice claims targeting the lack of neuromonitoring in CM cases suggests that SSEP neuromonitoring during CM may be unnecessary. We propose that neuromonitoring should not be used for routine CM decompression.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":"41 1","pages":"96"},"PeriodicalIF":1.3,"publicationDate":"2025-01-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143074030","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}
Cilmária Leite Franco, Jairo Porfirio de Oliveira Júnior, Bárbara Albuquerque Morais, Nayara Matos Pereira, Vicente Porfírio Pessoa Junior, Jordana Rodovalho Gontijo Germano, Ana Clara Tavares de Melo, Paulo Ronaldo Jubé Ribeiro
{"title":"Do routine antibiotics change the myelomeningocele infection rate? A case series.","authors":"Cilmária Leite Franco, Jairo Porfirio de Oliveira Júnior, Bárbara Albuquerque Morais, Nayara Matos Pereira, Vicente Porfírio Pessoa Junior, Jordana Rodovalho Gontijo Germano, Ana Clara Tavares de Melo, Paulo Ronaldo Jubé Ribeiro","doi":"10.1007/s00381-024-06748-3","DOIUrl":"10.1007/s00381-024-06748-3","url":null,"abstract":"<p><strong>Background: </strong>Myelomeningocele (MMC) is the most common type of congenital spinal malformation, typically requiring surgical intervention. While prenatal repair is increasingly favored, postnatal repair remains the standard in many settings. This study aims to evaluate the antibiotics prescribed to neonates with MMC and their correlation with central nervous system (CNS) infection rates following postnatal surgical repair.</p><p><strong>Methods: </strong>A retrospective cohort study was conducted, including newborns who underwent postnatal MMC repair between August 2017 and June 2021 at the Children's Hospital of Goiânia. The study examined variables related to the pregnancy and birth periods, as well as anatomical and neurosurgical factors. Neonates were categorized into four groups based on the time interval between birth and surgical repair (within 24, 48, 72 h, and beyond 72 h) and were evaluated for CNS infection rates.</p><p><strong>Results: </strong>A total of 38 neonates were included, with 24.32% diagnosed with CNS infections post-surgery. Notably, 22.86% of mothers did not receive prenatal care. Cesarean section was the predominant mode of delivery, accounting for 77.78% of cases. The average time from birth to surgery was 67 h. At admission, 73.68% of MMC cases presented with a rupture of the MMC membrane, and 78.94% of newborns received intravenous antibiotics. The mean surgical time was 60 min, with 78.94% of surgeries performed by experienced surgeons.</p><p><strong>Conclusion: </strong>Timely surgical intervention and the use of prophylactic antibiotics are critical in reducing CNS infection rates in neonates undergoing postnatal MMC repair. Delays in surgery and the lack of antibiotic prophylaxis were associated with higher infection rates, whereas factors such as delivery mode and surgeon experience had less impact on infection outcomes. These findings highlight the need for standardized care protocols and improved prenatal care to optimize neonatal outcomes.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":"41 1","pages":"95"},"PeriodicalIF":1.3,"publicationDate":"2025-01-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143051835","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}
Shi Hui Ong, Mervyn Jun Rui Lim, Char Loo Tan, Miriam Santiago Kimpo, Balamurugan A Vellayappan, Ai Peng Tan, Cynthia Hawkins, James Rutka, Vincent Diong Weng Nga
{"title":"Rare regression of congenital brainstem high-grade glioma: case report and literature review.","authors":"Shi Hui Ong, Mervyn Jun Rui Lim, Char Loo Tan, Miriam Santiago Kimpo, Balamurugan A Vellayappan, Ai Peng Tan, Cynthia Hawkins, James Rutka, Vincent Diong Weng Nga","doi":"10.1007/s00381-024-06658-4","DOIUrl":"https://doi.org/10.1007/s00381-024-06658-4","url":null,"abstract":"<p><p>Congenital infantile brainstem high-grade gliomas (HGGs) are extremely rare. Given the limited literature characterizing this disease, management of these tumors remains challenging. Brainstem HGGs are generally associated with extremely poor prognosis. Limited reports of spontaneous regression of radiologically diagnosed infantile brainstem tumors exist in published literature. In this case report, we document the first histologically proven congenital brainstem HGG with molecular characteristics that did not fall under any previously well-defined pediatric brain tumor classifications. The tumor underwent regression after biopsy, documented on neuroimaging up to 2 years of age. A review of the literature was also performed to identify previously reported infantile brainstem HGGs and the management for such tumors. Our case highlights the value of performing histopathological confirmation to guide management and the possible existence of a subcategory of a congenital brainstem HGG with better prognosis.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":"41 1","pages":"94"},"PeriodicalIF":1.3,"publicationDate":"2025-01-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143032430","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}
S M Stivaros, L M Parkes, R Bedir, E Cheesman, D Ram, L Leung, A Huang, J P Kilday
{"title":"Primary diffuse leptomeningeal atypical teratoid/rhabdoid tumours (ATRT) of childhood: a molecularly characterised case report and literature review.","authors":"S M Stivaros, L M Parkes, R Bedir, E Cheesman, D Ram, L Leung, A Huang, J P Kilday","doi":"10.1007/s00381-024-06698-w","DOIUrl":"10.1007/s00381-024-06698-w","url":null,"abstract":"<p><strong>Background: </strong>Atypical teratoid/rhabdoid tumours (ATRTs) are malignant central nervous system tumours, typically presenting in the posterior fossa of very young children. Prognosis remains poor despite current therapy, while tumorigenesis implicates both genomic and epigenetic dysregulation. Primary diffuse leptomeningeal (PDL) ATRT, characterised by the absence of an intraparenchymal mass lesion, is seldom reported but appears associated with a dismal outcome.</p><p><strong>Case presentation: </strong>We describe a 7-year-old male presenting with a PDL MYC-subgroup ATRT. The patient received multimodal upfront therapy, including high-dose craniospinal radiotherapy, embedded within a chemotherapy backbone. An unexpected clinical and radiological improvement was also observed upon cessation of all therapy for presumed disease progression. Although the patient eventually succumbed to the disease at 30 months, he demonstrated the longest survival for any PDL ATRT patient reported (median 8 months).</p><p><strong>Conclusion: </strong>Exhaustive literature review identified seven preceding published cases of PDL ATRT. Ours is the only one to have molecular subgrouping assigned. Perfusion imaging, within a multi-parametric diagnostic package, may be a sensitive marker for malignancy against other aetiologies in challenging presentations. Acknowledging the scarcity of the entity, we cautiously suggest a combination of chemotherapy and upfront high-dose craniospinal radiotherapy, if appropriate, may prolong survival for older children with PDL ATRT compared to exclusive chemotherapy or focal irradiation-based strategies. Our patient's recovery during palliation following a radiological diagnosis of disseminated relapse highlights the importance of confirming disease recurrence by tissue extraction where feasible.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":"41 1","pages":"93"},"PeriodicalIF":1.3,"publicationDate":"2025-01-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11754338/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143000927","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}