Bernd Schweiger

Purpose: The clinical relevance of low-grade intraventricular hemorrhage (IVH) in very preterm infants remains debated. We investigated how cMRI and cranial ultrasound (cUS) influence IVH detection and grading and how differences between the Papile and Volpe classifications affect interpretation and outcome stratification. Methods: This retrospective, single-center cohort study included 507 consecutively born infants at < 32 weeks’ gestation with serial cUS and cMRI at term-equivalent age (2009-2018). IVH was graded according to the Papile and Volpe classifications. Clinical parameters and neurodevelopmental outcomes at 24 months of corrected age were assessed (Bayley Scales of Infant Development). Univariable and multivariable regression analyses were performed. Results: Among 507 preterm infants, IVH was identified in 85 (16.8%; IVH I° n = 33, IVH II° n = 35, IVH III° n = 13, PVHI n = 4). CMRI and cUS differed significantly in IVH detection ( p = 0.025). IVH was not detected on cUS in 32 cases (37.6%, IVH I° n = 21, IVH II° n = 9, PVHI n = 2). Additionally, 48.6% of IVH II° according to Papile were classified as IVH I° on cUS due to non-detection of intraventricular blood. IVH II° was associated with significantly lower cognitive and motor outcomes scores than IVH I° ( p = 0.035/ p = 0.016). Differences in outcome stratification between grading systems were significant (cognitive p = 0.047/motor p = 0.015) and driven by differences in classification of intraventricular blood. Conclusion: Detection of intraventricular blood was associated with worse cognitive and motor outcomes. Differences between IVH grading systems and the choice of neuroimaging modality may influence risk stratification in infants with low-grade hemorrhage, particularly when small amounts of intraventricular blood are present.

Although the radiation dose impact of planning scans prior to the actual computed tomography (CT) scan has been addressed in previous studies, their systematic assessment across paediatric age groups and their relevance for establishing local diagnostic reference levels (DRLs) have not been thoroughly investigated and are not currently included in guideline recommendations. To investigate the influence of radiation dose from topogram, pre-monitoring, and monitoring scans by age on total radiation exposure and whether their doses need to be considered in DRLs and to establish local DRLs for these scan components. 102 chest CT scans with contrast material monitoring from 101 children (median age 12 years, interquartile range (IQR) 7–16 years) and 4498 chest CT scans from 4476 adults (⩾18 years) between January 2021 and May 2023 were retrospectively analysed. Paediatric scans were grouped into 5 age groups and compared with adult scans. The dose-length product (DLP) of topogram, pre-monitoring, and monitoring scans were evaluated and their effect on total radiation dose was analysed. Local DRLs were established for 5 different age groups. Radiation dose proportions of planning scans to total DLP in chest CT increased with decreasing age group (mainly p < 0.05), with the monitoring scan having the greatest impact. The median topogram proportion increased from 0.3% (⩾18 years) to 1.6% (<1 year), the pre-monitoring proportion from 0.5% (⩾18 years) to 3.5% (<1 year), and the monitoring proportion from 2.2% (⩾18 years) to 26.9% (<1 year). In children under 10 years, monitoring scans in chest CT have a large impact on radiation dose, contributing a median of 13%–27% of the total radiation dose compared to adults (median: 2.2%). Therefore, these planning scans should be monitored and included in radiation dose optimization efforts such as DRLs.

Purpose: In emergency diagnostics, head CT and CT angiography (CTA) of craniocervical vasculature are indispensable for children, despite their increased radiation sensitivity. This study assesses the radiation dose metrics of head CT and CTA in pediatric patients managed in the trauma resuscitation unit (TRU). Methods: All patients aged 0–<15 years who underwent head CT and CTA in the TRU between April 2020 and August 2023 were included. CT dose index volume (CTDIvol) and dose-length product (DLP) were extracted from the Radimetrics Enterprise Platform, which also provided organ doses estimated via Monte Carlo simulations and effective doses (ED) derived from these estimates. Dose metrics were compared with national diagnostic reference levels (DRLs), defined for three pediatric age groups: I (0–<5 years), II (5–<10 years), and III (10–<15 years). Results: Of 212 pediatric TRU patients, 62.7% (133/212) underwent CT and 72.2% (96/133) received combined CT and CTA. Median CTDIvol and DLP increased with age, whereas ED decreased. For head CT, CTDIvol ranged from 18.9 mGy to 29.4 mGy, DLP from 282 to 460 mGycm, and ED from 1.6 to 1.3 mSv. For CTA, CTDIvol ranged from 1.4 to 2.2 mGy, DLP from 40 to 83 mGycm, and ED from 1.0 to 0.8 mSv. All doses remained below national DRLs. Conclusion: Head CT and CTA in pediatric trauma can be performed with radiation doses well below national DRLs. Careful dose management is important to reduce potential long-term cancer risks and deterministic effects such as lens cataract formation.

Background: This study was conducted to evaluate the clinical applicability of integrated PET/MRI for staging and monitoring the effectiveness of neoadjuvant chemotherapy in Ewing sarcoma patients. Methods: A total of 11 juvenile patients with confirmed Ewing sarcoma, scheduled for induction polychemotherapy, were prospectively enrolled for a PET/MR examination before, during and after the end of treatment. Two experienced physicians analysed the imaging datasets. They were asked to perform a whole-body staging in all three examinations and to define treatment response according to the RECIST1.1 and PERCIST criteria for each patient. Results: In eight patients lymph node and/or distant metastases were detected at initial diagnosis. According to the reference standard, three patients achieved complete response, six patients partial response, and one patient showed stable disease while another patient showed progressive disease. RECIST1.1 categorized the response to treatment in 5/11 patients correctly and showed a tendency to underestimate the response to treatment in the remaining six patients. PERCIST defined response to treatment in 9/11 patients correctly and misclassified two patients with a PR as CR. Conclusion: PET/MRI may serve as a valuable imaging tool for primary staging and response assessment of juvenile patients with Ewing sarcoma to induction chemotherapy, accompanied by a reasonable radiation dose for the patient.

(1) Background: Immune–mediated necrotizing myopathy (IMNM) is a rare form of inflammatory muscle disease which is even more rare in pediatric patients. To increase the knowledge of juvenile IMNM, we here present the clinical findings on long-term follow-up, myopathological changes, and therapeutic strategies in two juvenile patients. (2) Methods: Investigations included phenotyping, determination of antibody status, microscopy on muscle biopsies, MRI, and response to therapeutic interventions. (3) Results: Anti-signal recognition particle (anti-SRP54) and anti- 3-hydroxy-3-methylglutarly coenzyme A reductase (anti-HMGCR) antibodies (Ab) were detected in the patients. Limb girdle presentation, very high CK-levels, and a lack of skin rash at disease-manifestation and an absence of prominent inflammatory signs accompanied by an abnormal distribution of α-dystroglycan in muscle biopsies initially hinted toward a genetically caused muscle dystrophy. Further immunostaining studies revealed an increase of proteins involved in chaperone-assisted autophagy (CASA), a finding already described in adult IMNM-patients. Asymmetrical muscular weakness was present in the anti-SRP54 positive Ab patient. After initial stabilization under therapy with intravenous immunoglobulins and methotrexate, both patients experienced a worsening of their symptoms and despite further therapy escalation, developed a permanent reduction of their muscle strength and muscular atrophy. (4) Conclusions: Diagnosis of juvenile IMNM might be complicated by asymmetric muscle weakness, lack of cutaneous features, absence of prominent inflammatory changes in the biopsy, and altered α-dystroglycan.

To improve the prediction of neurodevelopmental outcome in very preterm infants, this study used the combination of amplitude-integrated electroencephalography (aEEG) within the first 72 h of life and cranial magnetic resonance imaging (MRI) at term equivalent age. A single-center cohort of 38 infants born before 32 weeks of gestation was subjected to both investigations. Structural measurements were performed on MRI. Multiple regression analysis was used to identify independent factors including functional and structural brain measurements associated with outcome at a corrected age of 24 months. aEEG parameters significantly correlated with MRI measurements. Reduced deep gray matter volume was associated with low Burdjalov Score on day 3 (p < 0.0001) and day 1–3 (p = 0.0012). The biparietal width and the transcerebellar diameter were related to Burdjalov Score on day 1 (p = 0.0111; p = 0.0002). The final multiple regression analysis revealed independent predictors of neurodevelopmental outcome: intraventricular hemorrhage (p = 0.0060) and interhemispheric distance (p = 0.0052) for mental developmental index; Burdjalov Score day 1 (p = 0.0201) and interhemispheric distance (p = 0.0142) for psychomotor developmental index.

The purpose of this study was to evaluate the diagnostic accuracy of the sonographic fat pad sign (FPS) as a predictor for pediatric elbow fractures. This is a prospective study of children under 14 years with suspected elbow fractures. All participants underwent at first ultrasonography focused on a FPS followed by standard elbow radiographs. US findings were compared to final fracture diagnosis. 38 out of 79 children had an elbow fracture. A sonographic FPS predicted an elbow fracture with a sensitivity/specificity of 97.3/90.5 %, positive/negative likelihood ratios (LR) were 10.2/0.03 and correct/false classification rates were 93.7/6.3 %. Primary US findings were later reviewed by a blinded physician giving a congruity of 96.2 %. These secondary US findings predicted an elbow fracture with a sensitivity/specificity of 92.1/92.7 %, positive/negative LRs were 12.6/0.09 and correct/false classification rates were 92.4/7.5 %. The sonographic FPS could serve as a useful screening tool in primary evaluation of pediatric elbow injuries. If a fracture is unlikely after clinical and US evaluation, additional radiographs are dispensable, thereby potentially minimizing the radiation burden in childhood and reducing the length of stay in the Emergency Department.