Pediatric RadiologyPub Date : 2026-10-01Epub Date: 2026-07-22DOI: 10.1007/s00247-026-06734-8
Allison Lam, Bryce D Beutler, Siddhanth S Hegde, Matthew J Barkovich, Anthony R Zamary, Fernando A Torres
{"title":"Neuroimaging in FDXR-related mitochondriopathy.","authors":"Allison Lam, Bryce D Beutler, Siddhanth S Hegde, Matthew J Barkovich, Anthony R Zamary, Fernando A Torres","doi":"10.1007/s00247-026-06734-8","DOIUrl":"10.1007/s00247-026-06734-8","url":null,"abstract":"<p><p>Ferredoxin reductase (FDXR)-related mitochondriopathy is a rare autosomal recessive disorder caused by biallelic pathogenic variants in the FDXR gene. The clinical presentation is highly variable, ranging from isolated sensory neuropathies to severe infantile-onset encephalopathy with early demise. Neuroimaging is typically normal. However, oxidative stress in the setting of systemic infection can precipitate severe symptoms with distinct imaging manifestations, including confluent white matter signal abnormality, longitudinally extensive spinal cord lesions, and optic nerve enhancement. We describe a previously healthy and asymptomatic 2-year-old female with genetically confirmed FDXR-related mitochondriopathy who was found to have a distinct pattern of diffusion restriction, rapid parenchymal atrophy, and optic nerve changes following a respiratory illness. Early recognition and management of this rare but potentially devastating entity can help prevent vision loss and improve functional outcomes.</p>","PeriodicalId":19755,"journal":{"name":"Pediatric Radiology","volume":" ","pages":"2591-2595"},"PeriodicalIF":2.5,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13668887/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148550105","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pediatric RadiologyPub Date : 2026-10-01Epub Date: 2026-08-17DOI: 10.1007/s00247-026-06760-6
Mikhail Ya Yadgarov, Tamara L Antonevskaya, Yury N Likar
{"title":"<sup>18</sup>F-Metafluorobenzylguanidine positron emission tomography/computed tomography in neuroblastoma: reply to Albano et al.","authors":"Mikhail Ya Yadgarov, Tamara L Antonevskaya, Yury N Likar","doi":"10.1007/s00247-026-06760-6","DOIUrl":"10.1007/s00247-026-06760-6","url":null,"abstract":"","PeriodicalId":19755,"journal":{"name":"Pediatric Radiology","volume":" ","pages":"2427-2428"},"PeriodicalIF":2.5,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148796354","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"The effect of T2 signal heterogeneity on gadolinium enhancement in pediatric myelin oligodendrocyte glycoprotein antibody-associated disease: a quantitative magnetic resonance imaging study.","authors":"Lu Zhang, Xiao Fan, Wei-Qin Cheng, Shi-Yu Huang, Yin-Xin Liu, Wei Yu, Chuan Feng","doi":"10.1007/s00247-026-06694-z","DOIUrl":"10.1007/s00247-026-06694-z","url":null,"abstract":"<p><strong>Background: </strong>T2-hyperintensity and gadolinium enhancement mark inflammation in myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), yet the significance of T2 signal heterogeneity remains underexplored.</p><p><strong>Objective: </strong>To investigate the relationship between quantitative T2 signal heterogeneity metrics and lesion enhancement.</p><p><strong>Materials and methods: </strong>We retrospectively reviewed 70 MOGAD pediatric patients with 190 brain lesions (99 enhanced, 91 non-enhanced). Twenty-six patients underwent follow-up magnetic resonance imaging (MRI) (median 39 days) evaluating 57 baseline-enhanced lesions. Quantitative analysis included lesion area, T2 signal intensity (mean, standard deviation [SD], maximum [Max], minimum [Min]), and normalized ratios to the corpus callosum (CC).</p><p><strong>Results: </strong>Acute enhanced lesions exhibited larger axial areas and elevated T2 heterogeneity ratios (Max/CC, SD/CC, Max-Min/CC and Max/Min, all P<0.001) and Mean/CC (P=0.001) versus non-enhanced lesions. Follow-up lesions showed smaller areas (P<0.001) and normalized T2 ratios (Max/CC, SD/CC, Max-Min/CC and Max/Min, all P<0.001) versus their acute-phase counterparts. Analysis of covariance (ANCOVA) identified significant group×lesion area interaction effects for Max/CC, Max-Min/CC, and Max/Min (all P<0.05). Acute non-enhancing lesions and follow-up lesions showed no significant differences across all quantitative parameters (all P>0.05). The Max/Min ratio demonstrated superior diagnostic performance for lesion-level enhancement prediction (area under the receiver operating characteristic curve [AUC] 0.802, 95% confidence interval [CI] 0.739-0.864) and patient-level classification (AUC 0.837, 95% CI 0.739-0.934).</p><p><strong>Conclusion: </strong>T2 signal heterogeneity can suggest inflammatory activity in MOGAD. The Max/Min ratio provides a clinically applicable predictor for lesion enhancement and disease monitoring.</p>","PeriodicalId":19755,"journal":{"name":"Pediatric Radiology","volume":" ","pages":"2527-2536"},"PeriodicalIF":2.5,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148252632","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Third-window anomalies in children: an imaging-based educational review.","authors":"Melike Zeynep Akış, Fatma Ceren Sarıoğlu, Serhat Akış, Cemresu Akın, Enis Alpin Güneri, Handan Güleryüz Uçar","doi":"10.1007/s00247-026-06693-0","DOIUrl":"10.1007/s00247-026-06693-0","url":null,"abstract":"<p><p>Third-window anomalies represent a heterogeneous group of inner ear disorders characterized by abnormal openings in the bony otic capsule, resulting in altered cochlear and vestibular fluid mechanics. By creating an alternative pathway for inner ear fluid motion due to a new opening in addition to the oval and round windows, these lesions disrupt normal sound transmission and vestibular function. In pediatric patients, recognition of third-window anomalies is particularly important, as early and accurate diagnosis supports appropriate clinical assessment and management. High-resolution computed tomography of the temporal bone is the primary imaging modality for evaluating third-window anomalies, enabling detailed assessment of the bony labyrinth. However, many of these abnormalities are subtle and may be underestimated or misinterpreted on standard axial images. The use of anatomy-oriented multiplanar reconstructions tailored to the suspected defect significantly improves lesion conspicuity and diagnostic confidence. This educational review summarizes the anatomic and pathophysiologic principles underlying third-window phenomena in children and illustrates the imaging features of major entities, including semicircular canal dehiscence, enlarged vestibular aqueduct, and less common cochlear dehiscence. The aim is to provide a clear and practical imaging-based framework to support radiologists involved in the evaluation of pediatric inner ear abnormalities.</p>","PeriodicalId":19755,"journal":{"name":"Pediatric Radiology","volume":" ","pages":"2360-2375"},"PeriodicalIF":2.5,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13668931/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148259142","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pediatric RadiologyPub Date : 2026-10-01Epub Date: 2026-07-24DOI: 10.1007/s00247-026-06741-9
Kannan Sridharan, Gowri Sivaramakrishnan
{"title":"Potential value of incorporating full clinical data in vision-language model studies for pediatric appendicitis: reply to Mese et al.","authors":"Kannan Sridharan, Gowri Sivaramakrishnan","doi":"10.1007/s00247-026-06741-9","DOIUrl":"10.1007/s00247-026-06741-9","url":null,"abstract":"","PeriodicalId":19755,"journal":{"name":"Pediatric Radiology","volume":" ","pages":"2488"},"PeriodicalIF":2.5,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148579614","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Don't miss this treatable neurogenetic disease!","authors":"Matthew T Whitehead","doi":"10.1007/s00247-026-06796-8","DOIUrl":"https://doi.org/10.1007/s00247-026-06796-8","url":null,"abstract":"<p><p>Neurogenetic diseases are individually rare but collectively common. Recent advances in genetic diagnostics, expansion of newborn screening, and therapeutic progress make timely recognition of these disorders more crucial than ever. This review will encompass the most common white matter-dominant, gray matter-dominant, and magnetic resonance spectroscopy (MRS) characteristic genetic conditions causing metabolic disorders with (1) specific MRI/MRS features and (2) therapy available to cure, halt progression, slow progression, or reduce symptoms.</p>","PeriodicalId":19755,"journal":{"name":"Pediatric Radiology","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2026-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149081594","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ekaterina Korotkova, Sofya Kulikova, Olga Lvova, Dmitrii Tarasov, Alexey Meshkov, Olga Kovtun
{"title":"Methodological limitations of the BrainStructureAges pipeline from the volBrain platform: a brief report of the normative pediatric group.","authors":"Ekaterina Korotkova, Sofya Kulikova, Olga Lvova, Dmitrii Tarasov, Alexey Meshkov, Olga Kovtun","doi":"10.1007/s00247-026-06789-7","DOIUrl":"https://doi.org/10.1007/s00247-026-06789-7","url":null,"abstract":"<p><strong>Background: </strong>Predicted brain age is a widely used neuroimaging biomarker of brain maturation. However, limited normative pediatric datasets in model training raise concerns about systematic biases when applied to children. This study evaluates the hypothesis of predicted brain age overestimation in typically developing pediatric populations.</p><p><strong>Objective: </strong>To analyze predicted brain age and brain delta in a large, age- and sex-matched sample of typically developing children and to test whether there is any potential systematic overestimation of predicted brain age in children using the BrainStructureAges pipeline (LaBRI, CNRS/University of Bordeaux, Bordeaux, France).</p><p><strong>Materials and methods: </strong>We estimated predicted brain age from 105 brain MRI scans of healthy and typically developing children aged 6-9 years with well-documented medical histories. All scans were performed on a Magnetom Amira 1.5-T scanner (Siemens Healthineers, Erlangen, Germany), using T1-weighted MPRAGE sequences. Brain age prediction utilized the BrainStructureAges pipeline on the volBrain (ITACA Institute, Universitat Politècnica de València, Valencia, Spain, and LaBRI, CNRS/University of Bordeaux, Bordeaux, France) platform. The brain delta was defined as the difference between chronological age and predicted brain age. Regional estimates were obtained for 133 brain structures.</p><p><strong>Results: </strong>The mean predicted brain age was 9.3±1.2 years, compared to a chronological age of 7.7±0.4 years, resulting in a mean brain delta of 1.6±1.2 years. No significant sex differences were observed for chronological age, predicted brain age, or delta (boys, 1.55 years; girls, 1.61 years; t=0.27, P=0.79). Positive brain delta values were seen in 95.2% of participants across all structures, with the largest deviations in the left cerebellar exterior (+3.3 years), left occipital fusiform gyrus (+3.3 years), cerebellar lobules VI-VII (+2.9 years), left inferior occipital gyrus (+2.9 years), and right frontal pole (+2.8 years).</p><p><strong>Conclusions: </strong>These findings suggest a systematic overestimation of predicted brain age in children, highlighting the need for caution in clinical interpretations to prevent misinterpretations in pediatric populations.</p>","PeriodicalId":19755,"journal":{"name":"Pediatric Radiology","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2026-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149081586","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Carlos Yaya-Quezada, Jie C Nguyen, Julian Forero-Millan, Samuel S Hailu, Wondwossen T Lerebo, Benjamin H Taragin
{"title":"Distal humeral physeal separation: radiographic findings in infants and toddlers.","authors":"Carlos Yaya-Quezada, Jie C Nguyen, Julian Forero-Millan, Samuel S Hailu, Wondwossen T Lerebo, Benjamin H Taragin","doi":"10.1007/s00247-026-06790-0","DOIUrl":"https://doi.org/10.1007/s00247-026-06790-0","url":null,"abstract":"<p><strong>Background: </strong>Distal humeral physeal separation is an uncommon but frequently misdiagnosed injury of the immature elbow in infants and toddlers that carries a strong association with non-accidental trauma; its largely unossified anatomy makes radiographic diagnosis challenging.</p><p><strong>Objective: </strong>To evaluate radiographic features that can aid in the diagnosis of distal humeral physeal separation among infants and toddlers.</p><p><strong>Materials and methods: </strong>This retrospective comparative study included 19 children under 3 years of age with clinically or surgically confirmed distal humeral physeal separation and available pretreatment elbow and forearm radiographs, identified from the imaging database of a tertiary-care children's hospital (January 2008 to December 2024). Thirty-two age- and sex-matched control children without elbow fracture were selected from the same source population. Two radiologists independently reviewed all examinations, blinded to fracture status, and recorded the presence or absence of soft tissue swelling, joint effusion, abnormal ossific fragmentation, and abnormal medial or posterior forearm alignment. Findings were compared between children with and without separation injury.</p><p><strong>Results: </strong>This study group included 51 children (32 boys, 19 girls; median age, 17 days, range, 0-1186 days). Soft-tissue swelling (anteroposterior: 94.7% vs 34.4%, P<.001; lateral: 78.9% vs 12.5%, P<.001), abnormal ossific fragmentation (78.9% vs 3.1%, P<.001), and abnormal forearm alignment (medial: 89.5% vs 0%, P<.001; posterior: 78.6% vs 0%, P<.001) were more common in children with physeal separation than those without. On multivariable analysis, abnormal medial forearm alignment remained associated with physeal separation (OR=104.2; 95% CI, 3.9-2775.5; P=.001).</p><p><strong>Conclusion: </strong>In our study group, several radiographic findings were associated with distal humeral physeal separation in infants and toddlers; however, only abnormal medial forearm alignment remained strongly associated with physeal separation.</p>","PeriodicalId":19755,"journal":{"name":"Pediatric Radiology","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2026-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149063443","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}