Search

Introduction: Many chronic pediatric liver disorders are complicated by the development of fibrosis and ultimately cirrhosis. Although hepatic fibrogenesis progresses along a common pathway irrespective of the specific etiology, fibrosis in pediatric liver diseases has different histopathological patterns than in adults. In pediatric liver disease, as in adults, management choices may depend upon the stage of fibrosis at diagnosis. With early intervention, the progression of hepatic fibrosis can be slowed or halted, and in some situations, reversed. While liver biopsy is the gold standard for diagnosing and assessing the presence and degree of fibrosis, it has several disadvantages including the potential for sampling error, the risk of complications, the relatively high cost, and general poor acceptance by pediatric patients and their parents. MR elastography (MRE) is a relatively new imaging technique with the potential for allowing a safe, rapid, cost-effective, and non-invasive evaluation of a wide variety of hepatic diseases by quantitatively evaluating the stiffness of the liver parenchyma. The purpose of this article is to present our initial clinical experience and illustrate our modified technique for the application of liver MRE in pediatric patients at our medical center.Methods and Materials: Pediatric MRE techniques were developed and applied to over 45 patients scanned with our new protocol.Conclusion: Liver MRE is a safe, non-invasive method for assessing hepatic fibrosis in pediatric patients. [ABSTRACT FROM AUTHOR]

Gadoxetate disodium (Gd-EOB-DTPA) is a hepatobiliary MRI contrast agent widely used in adults for characterization of liver tumors and increasingly used in children. Hepatoblastoma is the most common primary hepatic malignancy of childhood. In this review, we describe our experience with this agent both before and after initiating therapy in children with hepatoblastoma. [ABSTRACT FROM AUTHOR]

Background: Pancreatic duct (PD) or common bile duct (CBD) dilatation can indicate ductal pathology, but limited data describe normal pediatric duct measurements on routine 2D MR sequences. Objective: To characterize the visibility and diameter of the PD and CBD on 2D MR images in children without pancreaticobiliary disease. Methods: This retrospective study included patients who underwent abdominal MRI using a rapid protocol (comprised of noncontrast axial and coronal 2D SSFSE sequences) to assess for suspected appendicitis or ovarian torsion in the emergency department setting between January 23, 2023, and September 13, 2023, excluding patients with a pancreatic or hepatobiliary abnormality on MRI or laboratory assessment. Four radiologists independently reviewed examinations. Reviewers recorded PD visibility in each of four segments (head, neck, body, and tail) and CBD visibility, and measured PD diameter in each segment and maximal CBD diameter. Duct measurements by age were characterized by linear regression analyses. Results: The study included 177 patients [112 female, 65 female; mean age, 12.3±3.4 years (range, 5.1-17.7 years)]. The observers reported PD visibility in the head in 32.5-93.5%, neck in 18.4-71.5%, body in 22.3-69.8%, and tail in 7.3-25.7%, and in all four segments in 6.2-22.4%, of patients. Maximum PD diameter in any segment, as a mean across observers, was 1.8 mm (range across observers, 0.7-3.5 mm). Expected maximal PD diameter in any segment, in terms of the 5th and 95th percentile values of observers' mean measurements, was 1.4-2.3 mm; the prediction interval's upper limit increased from age 5 to 17 from 2.1 to 2.5 mm. All observers reported CBD visibility in all patients. Mean CBD diameter across observers was 3.1 mm (range across observers, 2.9-3.4 mm). Expected CBD diameter, in terms of the 5th and 95th percentile values of observers' mean measurements, was 2.3-4.9 mm; the prediction interval's upper limit increased from age 5 to 17 from 3.9 to 5.0 mm. Conclusion: We report expected upper limits for PD and CBD measurements on 2D MR images in children without evidence of pancreaticobiliary disease. Clinical Impact: These findings may aid radiologists' identification of pancreaticobiliary duct abnormalities on routine abdominal MRI examinations.

Background: Despite advances in medical therapy, many children and adults with ileal Crohn's disease (CD) progress to fibrostenosis requiring surgery. We aimed to identify MRI and circulating biomarkers associated with the need for surgical management., Methods: This prospective, multicenter study included pediatric and adult CD cases undergoing ileal resection and CD controls receiving medical therapy. Noncontrast research MRI examinations measured bowel wall 3-dimensional magnetization transfer ratio normalized to skeletal muscle (normalized 3D MTR), modified Look-Locker inversion recovery (MOLLI) T1 relaxation, intravoxel incoherent motion (IVIM) diffusion-weighted imaging metrics, and the simplified magnetic resonance index of activity (sMaRIA). Circulating biomarkers were measured on the same day as the research MRI and included CD64, extracellular matrix protein 1 (ECM1), and granulocyte-macrophage colony-stimulating factor (GM-CSF) autoantibodies (Ab). Associations between MRI and circulating biomarkers and need for ileal resection were tested using univariate and multivariable LASSO regression., Results: Our study sample included 50 patients with CD undergoing ileal resection and 83 patients with CD receiving medical therapy; mean participant age was 23.9 ± 13.1 years. Disease duration and treatment exposures did not vary between the groups. Univariate biomarker associations with ileal resection included log GM-CSF Ab (odds ratio [OR], 2.87; P = .0009), normalized 3D MTR (OR, 1.05; P = .002), log MOLLI T1 (OR, 0.01; P = .02), log IVIM perfusion fraction (f; OR, 0.38; P = .04), and IVIM apparent diffusion coefficient (ADC; OR, 0.3; P = .001). The multivariable model for surgery based upon corrected Akaike information criterion included age (OR, 1.03; P = .29), BMI (OR, 0.91; P = .09), log GM-CSF Ab (OR, 3.37; P = .01), normalized 3D MTR (OR, 1.07; P = .007), sMaRIA (OR, 1.14; P = .61), luminal narrowing (OR, 10.19; P = .003), log C-reactive protein (normalized; OR, 2.75; P = .10), and hematocrit (OR, 0.90; P = .13)., Conclusion: After accounting for clinical and MRI measures of severity, normalized 3D MTR and GM-CSF Ab are associated with the need for surgery in ileal CD., (© The Author(s) 2024. Published by Oxford University Press on behalf of Crohn’s & Colitis Foundation. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.)

Background: MRI diffusion-weighted imaging (DWI) is commonly used in MR enterography protocols for assessment of intestinal inflammation in patients with Crohn's disease. The intravoxel incoherent motion (IVIM) approach to DWI has been proposed as a more objective approach, providing quantitative parameters that reflect water diffusivity (D), blood flow (D*), and perfusion fraction (f)., Purpose: We aimed to determine if DWI-IVIM metrics from the terminal ileum in patients with newly diagnosed Crohn's disease differ from healthy participants and change in response to biologic medical therapy., Methods: In this prospective case-control study, 20 consecutive pediatric patients (mean age = 14 years ± 2 [SD]; eight females) with newly diagnosed ileal Crohn's disease and 15 pediatric healthy participants (mean age = 18 years ± 4 [SD]; eight females) underwent research MRI examinations of the small bowel between 12/2018 and 10/2021. Participants with Crohn's disease underwent MR studies at baseline, 6 weeks, and 6 months following initiation of anti-TNF-alpha therapy, whereas control participants underwent one research MRI examination. The MRI protocol included a DWI-IVIM sequence with nine b-values and the IVIM parameters (D, D*, and f) were extracted. Unpaired t-tests and mixed-effects models were used for analyses., Results: Mean IVIM D (P < 0.001), D* (P = 0.004), and f (P = 0.001) metrics were lower for Crohn's patients at the time of diagnosis compared to healthy participants. Mean IVIM f value increased over time in response to medical therapy (mean f at baseline, 22% ± 6%; 6 weeks, 25% ± 7%; 6 months, 29% ± 10%; P = 0.016). Mean IVIM D* value increased over time in response to treatment (mean D* at baseline, 10.9 ± 3.0 × 10 -3 mm 2 /s; 6 weeks, 11.8 ± 2.8 × 10 -3 mm 2 /s; 6 months, 13.3 ± 3.3 × 10 -3 mm 2 /s; P = 0.047), while there was no significant change in mean IVIM D value (P = 0.10)., Conclusion: MRI DWI-IVIM metrics in patients with ileal Crohn's disease change over time in response to biological therapy and help discriminate these patients from healthy participants., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Purpose: (1) To determine the frequency of surgical management in children with Crohn's Disease (CD) and a new radiologic ileal stricture, and (2) to identify imaging and clinical features that predict the need for surgery., Methods: This retrospective study included pediatric patients (< 21 years old) with CD and a new ileal stricture diagnosed by MRE, CTE, or CT between July 2018 and June 2023. Three board-certified radiologists recorded stricture length, maximum mural thickness, minimum lumen diameter, maximum upstream diameter, and simplified magnetic resonance index of activity (sMaRIA) score. Anthropometrics, laboratory data, and surgical interventions performed after stricture diagnosis were also recorded. Multivariable logistic regression was used to identify imaging and clinical variables associated with the need for surgery., Results: 44 pediatric CD patients (median age 16.5 years) presented with a new ileal stricture during the study period. 30 (68.2%) patients required surgery, with a median time of 87.5 days between stricture diagnosis and surgery. Median stricture measurements were length: 7.0 cm, maximum mural thickness: 7.3 mm, minimum lumen diameter: 0.2 cm, and maximum upstream diameter: 3.3 cm. Median sMaRIA score was 3.0, and 14 (31.8%) strictures had associated internal penetrating disease. Stricture ratio (ratio of maximum upstream lumen diameter to minimum lumen diameter) (OR = 1.15 [95% CI 1.02-1.30]; p = 0.02) and sMaRIA (OR = 2.12 [95% CI 0.87-5.17; p = 0.10) were associated with need for surgery., Conclusion: Surgery remains common in stricturing pediatric CD, with increasing stricture ratio and sMaRIA score associated with need for surgical management., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Sensitive images represent a new challenge in enterprise imaging. These images, often containing nudity or gruesome content, have the potential to cause emotional harm to patients and people who view the images. Unfortunately, the interoperability standards used in imaging informatics have not yet addressed this issue. Because of this, the software solutions used in healthcare information technology are not able to offer patients and other viewers of image protections. In this Health Information Management Systems Society (HIMSS)/Society for Imaging Informatics in Medicine (SIIM) Enterprise Imaging Community Whitepaper, we define sensitive images, identify unique challenges related to their management, and provide recommendations for future solutions to protect our patients., (© 2024. The Author(s).)

Purpose: To assess ChatGPT's accuracy, relevance, and readability in answering patients' common imaging-related questions and examine the effect of a simple prompt., Methods: A total of 22 imaging-related questions were developed from categories previously described as important to patients, as follows: safety, the radiology report, the procedure, preparation before imaging, meaning of terms, and medical staff. These questions were posed to ChatGPT with and without a short prompt instructing the model to provide an accurate and easy-to-understand response for the average person. Four board-certified radiologists evaluated the answers for accuracy, consistency, and relevance. Two patient advocates also reviewed responses for their utility for patients. Readability was assessed using the Flesch Kincaid Grade Level. Statistical comparisons were performed using χ 2 and paired t tests., Results: A total of 264 answers were assessed for both unprompted and prompted questions. Unprompted responses were accurate 83% of the time (218 of 264), which did not significantly change for prompted responses (87% [229 of 264]; P = .2). The consistency of the responses increased from 72% (63 of 88) to 86% (76 of 88) when prompts were given (P = .02). Nearly all responses (99% [261 of 264]) were at least partially relevant for both question types. Fewer unprompted responses were considered fully relevant at 67% (176 of 264), although this increased significantly to 80% when prompts were given (210 of 264; P = .001). The average Flesch Kincaid Grade Level was high at 13.6 [CI, 12.9-14.2], unchanged with the prompt (13.0 [CI, 12.41-13.60], P = .2). None of the responses reached the eighth-grade readability level recommended for patient-facing materials., Discussion: ChatGPT demonstrates the potential to respond accurately, consistently, and relevantly to patients' imaging-related questions. However, imperfect accuracy and high complexity necessitate oversight before implementation. Prompts reduced response variability and yielded more-targeted information, but they did not improve readability. ChatGPT has the potential to increase accessibility to health information and streamline the production of patient-facing educational materials; however, its current limitations require cautious implementation and further research., (Copyright © 2023. Published by Elsevier Inc.)

Introduction: We evaluate the role of apparent diffusion coefficient (ADC) histogram metrics in stratifying pediatric and young adult rhabdomyosarcomas., Methods: We retrospectively evaluated baseline diffusion-weighted imaging (DWI) from 38 patients with rhabdomyosarcomas (Not otherwise specified: 2; Embryonal: 21; Spindle Cell: 2; Alveolar: 13, mean ± std dev age: 8.1 ± 7.76 years). The diffusion images were obtained on a wide range of 1.5 T and 3 T scanners at multiple sites. FOXO1 fusion status was available for 35 patients, nine of whom harbored the fusion. 13 patients were TNM stage 1, eight had stage 2 disease, nine were stage 3, and eight had stage 4 disease. 23 patients belonged to Clinical Group III and seven to Group IV, while two and five were CG I and II, respectively. Nine patients were classified as low risk, while 21 and five were classified as intermediate and high risk respectively. Histogram parameters of the apparent diffusion coefficient (ADC) map from the entire tumor were obtained based on manual tumor contouring. A two-tailed Mann-Whitney U test was used for all two-group, and the Kruskal-Wallis's test was used for multiple-group comparisons. Bootstrapped receiver operating characteristic (ROC) curves and areas under the curve (AUC) were generated for the statistically significant histogram parameters to differentiate genotypic and phenotypic parameters., Results: Alveolar rhabdomyosarcomas had a statistically significant lower 10th Percentile (586.54 ± 164.52, mean ± std dev, values are in ×10-6mm 2 /s) than embryonal rhabdomyosarcomas (966.51 ± 481.33) with an AUC of 0.85 (95%CI. 0.73-0.95) for differentiating the two. The 10th percentile was also significantly different between FOXO1 fusion-positive (553.87 ± 187.64) and negative (898.07 ± 449.38) rhabdomyosarcomas with an AUC of 0.83 (95% CI 0.71-0.94). Alveolar rhabdomyosarcomas also had statistically significant lower Mean, Median, and Root Mean Squared ADC histogram values than embryonal rhabdomyosarcomas. Four, five, and seven of the 18 histogram parameters evaluated demonstrated a statistically significant increase with higher TNM stage, clinical group, assignment, and pretreatment risk stratification, respectively. For example, Entropy had an AUC of 0.8 (95% CI. 0.67-0.92) for differentiating TNM stage 1 from ≥ stage 2 and 0.9 (95% CI. 0.8-0.98) for differentiating low from intermediate or high-risk stratification., Conclusion: Our findings demonstrate the potential of ADC histogram metrics to predict clinically relevant variables for rhabdomyosarcoma, including FOXO1 fusion status, histopathology, Clinical Group, TNM staging, and risk stratification., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Association of University Radiologists. Published by Elsevier Inc. All rights reserved.)

Objective: To evaluate the estimated labor costs and effectiveness of Ongoing Professional Practice Evaluation (OPPE) processes at identifying outlier performers in a large sample of providers across multiple health care systems and to extrapolate costs and effectiveness nationally., Methods: Six hospital systems partnered to evaluate their labor expenses related to conducting OPPE. Estimates for mean labor hours and wages were created for the following: data analysts, medical staff office professionals, department physician leaders, and administrative assistants. The total number of outlier performers who were identified by OPPE metrics alone and that resulted in lack of renewal, limitation, or revoking of hospital privileges during the past annual OPPE cycle (2022) was recorded. National costs of OPPE were extrapolated. Literature review of the effect of OPPE on safety culture in radiology was performed., Results: The evaluated systems had 12,854 privileged providers evaluated by OPPE. The total estimated annual recurring labor cost per provider was $50.20. Zero of 12,854 providers evaluated were identified as outlier performers solely through the OPPE process. The total estimated annual recurring cost of administering OPPE nationally was $78.54 million. In radiology over the past 15 years, the use of error rates based on score-based peer review as an OPPE metric has been perceived as punitive and had an adverse effect on safety culture., Conclusion: OPPE is expensive to administer, inefficient at identifying outlier performers, diverts human resources away from potentially more effective improvement work, and has been associated with an adverse impact on safety culture in radiology., (Copyright © 2023 American College of Radiology. Published by Elsevier Inc. All rights reserved.)

Background: Neuroblastoma risk stratification relies on prognostic risk factors and image-defined risk factors (IDRFs). Evaluating neuroblastoma typically involves magnetic resonance imaging (MRI) with gadolinium-based contrast media (GBCM, "contrast"). However, there are concerns regarding adverse effects and cost of GBCM. We aimed to assess the impact of intravenous GBCM on interobserver agreement for neuroblastoma staging on baseline MRI., Procedure: We reviewed baseline MRI scans of 50 children with abdominopelvic neuroblastomas confirmed by histopathology. Duplicate sets of images were created, with post-contrast T1-weighted sequences removed from one set. Four pediatric radiologists independently analyzed the scans in a randomized manner. They recorded primary tumor size, presence of IDRFs, and metastatic lesions. Agreement among the reviewers was measured using kappa and Fleiss kappa statistics., Results: Mean age of included children was 3.3 years (range: 0.01-14.9 years), and 20 [40%] were females. Mean tumor size was 5.7 cm in greatest axial diameter. Pre-contrast versus post-contrast MRI showed excellent agreement for tumor measurement. Overlapping confidence intervals (CIs) were seen in nearly all categories of interobserver agreement on the presence or absence of individual IDRFs, with agreement ranging from poor to substantial, regardless of the presence of contrast. The overall interobserver agreement on the presence of at least one IDRF was substantial with contrast (kappa = .63; 95% CI: .52-.75) and moderate without contrast (kappa = .5; 95% CI: .39-.61); although the overlapping CIs suggest a lack of meaningful difference. Similarly, interobserver agreement on the presence or absence of individual sites of metastatic disease ranged from poor to substantial. The interobserver agreement on the overall determination of presence of metastatic disease was fair with contrast (kappa = .49; 95% CI: .38-.61) and moderate without contrast (kappa = .71; 95% CI: .59-.826)., Conclusions: Contrast does not improve tumor size measurement or radiologist agreement on the presence or absence of IDRFs or metastatic disease in children with newly diagnosed neuroblastoma., (© 2023 Wiley Periodicals LLC.)

BACKGROUND. Radiologists have variable diagnostic performance and considerable interreader variability when interpreting MR enterography (MRE) examinations for suspected Crohn disease (CD). OBJECTIVE. The purposes of this study were to develop a machine learning method for predicting ileal CD by use of radiomic features of ileal wall and mesenteric fat from noncontrast T2-weighted MRI and to compare the performance of the method with that of expert radiologists. METHODS. This single-institution study included retrospectively identified patients who underwent MRE for suspected ileal CD from January 1, 2020, to January 31, 2021, and prospectively enrolled participants (patients with newly diagnosed ileal CD or healthy control participants) from December 2018 to October 2021. Using axial T2-weighted SSFSE images, a radiologist selected two slices showing greatest terminal ileal wall thickening. Four ROIs were segmented, and radiomic features were extracted from each ROI. After feature selection, support-vector machine models were trained to classify the presence of ileal CD. Three fellowship-trained pediatric abdominal radiologists independently classified the presence of ileal CD on SSFSE images. The reference standard was clinical diagnosis of ileal CD based on endoscopy and biopsy results. Radiomic-only, clinical-only, and radiomic-clinical ensemble models were trained and evaluated by nested cross-validation. RESULTS. The study included 135 participants (67 female, 68 male; mean age, 15.2 ± 3.2 years); 70 were diagnosed with ileal CD. The three radiologists had accuracies of 83.7% (113/135), 88.1% (119/135), and 86.7% (117/135) for diagnosing CD; consensus accuracy was 88.1%. Interradiologist agreement was substantial (κ = 0.78). The best-performing ROI was bowel core (AUC, 0.95; accuracy, 89.6%); other ROIs had worse performance (whole-bowel AUC, 0.86; fat-core AUC, 0.70; whole-fat AUC, 0.73). For the clinical-only model, AUC was 0.85 and accuracy was 80.0%. The ensemble model combining bowel-core radiomic and clinical models had AUC of 0.98 and accuracy of 93.5%. The bowel-core radiomic-only model had significantly greater accuracy than radiologist 1 ( p = .009) and radiologist 2 ( p = .02) but not radiologist 3 ( p > .99) or the radiologists in consensus ( p = .05). The ensemble model had greater accuracy than the radiologists in consensus ( p = .02). CONCLUSION. A radiomic machine learning model predicted CD diagnosis with better performance than two of three expert radiologists. Model performance improved when radiomic data were ensembled with clinical data. CLINICAL IMPACT. Deployment of a radiomic-based model including T2-weighted MRI data could decrease interradiologist variability and increase diagnostic accuracy for pediatric CD.

The incorporation of artificial intelligence into radiological clinical workflow is on the verge of being realized. To ensure that these tools are effective, measures must be taken to educate radiologists on tool performance and failure modes. Additionally, radiology systems should be designed to avoid automation bias and the potential decline in radiologist performance. Designed solutions should cater to every level of expertise so that patient care can be enhanced and risks reduced. Ultimately, the radiology community must provide education so that radiologists can learn about algorithms, their inputs and outputs, and potential ways they may fail. This manuscript will present suggestions on how to train radiologists to use these new digital systems, how to detect AI errors, and how to maintain underlying diagnostic competency when the algorithm fails., (© 2023. The Author(s) under exclusive licence to Society for Imaging Informatics in Medicine.)

Background: Pleuropulmonary blastoma (PPB) is the most common primary lung neoplasm of infancy and early childhood. Given the rarity of PPB, the role of positron emission tomography (PET) and bone scintigraphy (bone scans) in diagnostic evaluation and surveillance has not been documented to date. Available PET and bone scan data are presented in this study., Procedures: Patients with PPB enrolled in the International PPB/DICER1 Registry and available PET imaging and/or bone scan reports were retrospectively abstracted., Results: On retrospective analysis, 133 patients with type II and III (advanced) PPB were identified with available report(s) (PET scan only = 34, bone scan only = 83, and both bone scan and PET = 16). All advanced primary PPB (n = 11) and recurrent (n = 8) tumors prior to treatment presented with 18 F-fluorodeoxyglucose (FDG)-avid lesions, with median maximum standardized uptake values of 7.4 and 6.7, respectively. False positive FDG uptake in the thorax was noted during surveillance (specificity: 59%). Bone metastases were FDG-avid prior to treatment. Central nervous system metastases were not discernable on PET imaging. Sensitivity and specificity of bone scans for metastatic bone disease were 89% and 92%, respectively. Bone scans had a negative predictive value of 99%, although positive predictive value was 53%. Four patients with distant bone metastases had concordant true positive bone scan and PET., Conclusion: Primary, recurrent, and/or extracranial metastatic PPB presents with an FDG-avid lesion on PET imaging. Additional prospective studies are needed to fully assess the utility of nuclear medicine imaging in surveillance for patients with advanced PPB., (© 2023 Wiley Periodicals LLC.)

Background: Pediatric radiologists can identify a liver ultrasound (US) pattern predictive of progression to advanced liver disease. However, reliably discriminating these US patterns remains difficult. Quantitative magnetic resonance imaging (MRI) may provide an objective measure of liver disease in cystic fibrosis (CF)., Objective: The purpose of this study was to determine if quantitative MRI, including MR elastography, is feasible in children with CF and to determine how quantitative MRI-derived metrics compared to a research US., Materials and Methods: A prospective, multi-institutional trial was performed evaluating CF participants who underwent a standardized MRI. At central review, liver stiffness, fat fraction, liver volume, and spleen volume were obtained. Participants whose MRI was performed within 1 year of US were classified by US pattern as normal, homogeneous hyperechoic, heterogeneous, or nodular. Each MRI measure was compared among US grade groups using the Kruskal-Wallis test., Results: Ninety-three participants (51 females [54.8%]; mean 15.6 years [range 8.1-21.7 years]) underwent MRI. MR elastography was feasible in 87 participants (93.5%). Fifty-eight participants had an US within 1 year of MRI. In these participants, a nodular liver had significantly higher stiffness (P<0.01) than normal or homogeneous hyperechoic livers. Participants with a homogeneous hyperechoic liver had a higher fat fraction (P<0.005) than others., Conclusion: MR elastography is feasible in children with CF. Participants with a nodular pattern had higher liver stiffness supporting the US determination of advanced liver disease. Participants with a homogeneous hyperechoic pattern had higher fat fractions supporting the diagnosis of steatosis., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

The liver is the primary organ for the metabolism of many chemotherapeutic agents. Treatment-induced liver injury is common in children undergoing cancer therapy. Hepatic injury occurs due to various mechanisms, including biochemical cytotoxicity, hepatic vascular injury, radiation-induced cytotoxicity, and direct hepatic injury through minimally invasive and invasive surgical treatments. Treatment-induced liver injury can be seen contemporaneous with therapy and months to years after therapy is complete. Patients can develop a combination of hepatic injuries manifesting during and after treatment. Acute toxic effects of cancer therapy in children include hepatitis, steatosis, steatohepatitis, cholestasis, hemosiderosis, and vascular injury. Longer-term effects of cancer therapy include hepatic fibrosis, chronic liver failure, and development of focal liver lesions. Quantitative imaging techniques can provide useful metrics for disease diagnosis and monitoring, especially in treatment-related diffuse liver injury such as hepatic steatosis and steatohepatitis, hepatic iron deposition, and hepatic fibrosis. Focal liver lesions, including those developing as a result of treatment-related vascular injury such as focal nodular hyperplasia-like lesions and hepatic perfusion anomalies, as well as hepatic infections occurring as a consequence of immune suppression, can be anxiety provoking and confused with recurrent malignancy or hepatic metastases, although there often are imaging features that help elucidate the correct diagnosis. Radiologic evaluation, in conjunction with clinical and biochemical screening, is integral to diagnosing and monitoring hepatic complications of cancer therapy in pediatric patients during therapy and after therapy completion for long-term surveillance. © RSNA, 2023 Quiz questions for this article are available in the supplemental material See the invited commentary by Ferraciolli and Gee in this issue.

In this white paper, the ACR Pediatric AI Workgroup of the Commission on Informatics educates the radiology community about the health equity issue of the lack of pediatric artificial intelligence (AI), improves the understanding of relevant pediatric AI issues, and offers solutions to address the inadequacies in pediatric AI development. In short, the design, training, validation, and safe implementation of AI in children require careful and specific approaches that can be distinct from those used for adults. On the eve of widespread use of AI in imaging practice, the group invites the radiology community to align and join Image IntelliGently (www.imageintelligently.org) to ensure that the use of AI is safe, reliable, and effective for children., (Copyright © 2023 American College of Radiology. Published by Elsevier Inc. All rights reserved.)

Background: Hepatoblastoma (HB) is the most common liver malignancy in children. There is no standard of care for management of relapsed/refractory HB (rrHB) and reports in the literature are limited., Objective: To describe presenting features, biology, treatment strategies, and outcomes for pediatric patients with relapsed/refractory hepatoblastoma., Methods: An IRB-approved retrospective institutional review of patients with rrHB who presented for consultation and/or care from 2000-2019. Clinical, radiographic, and histologic data were collected from all patients., Results: Thirty subjects were identified with a median age of 19.5 months (range 3-169 months) at initial diagnosis and 32.5 months (range 12-194 months) at time of first relapse. 63% of subjects were male, 70% Caucasian, and 13% were born premature. Three subjects had a known cancer predisposition syndrome. Eight patients had refractory disease while 22 patients had relapsed disease. Average time from initial diagnosis to relapse or progression was 12.5 months. Average alpha-fetoprotein (AFP) at initial diagnosis was 601,203 ng/mL (range 121-2,287,251 ng/mL). Average AFP at relapse was 12,261 ng/mL (range 2.8-201,000 ng/mL). For patients with tumor sequencing ( n = 17), the most common mutations were in CTNNB1 (13) and NRF2 (4). First relapse sites were lungs ( n = 12), liver ( n = 11) and both ( n = 6). More than one relapse/progression occurred in 47% of subjects; 6 had ≥3 relapses. Pathology in patients with multiply relapsed disease was less differentiated including descriptions of small cell undifferentiated ( n = 3), pleomorphic ( n = 1), transitional liver cell tumor ( n = 2) and HB with carcinoma features ( n = 1). All subjects underwent surgical resection of site of relapsed disease with 7 subjects requiring liver transplantation. Overall survival was 50%. Survival was associated with use of cisplatin at relapse (78.6% with vs. 25% without, p = 0.012). The most common late effect was ototoxicity with at least mild sensorineural hearing loss found in 80% of subjects; 54% required hearing aids., Conclusions: Retreatment with cisplatin at the time of relapse may provide an advantage for some patients with hepatoblastoma. Multiply relapsed disease was not uncommon and not associated with a worse prognosis. Careful attention should be paid to cumulative therapy-induced toxicity while concurrently aiming to improve cure.

BACKGROUND. Contrast-enhanced MRI is commonly used to evaluate thoracic central venous patency in children and young adults. A flow-independent noncontrast non-ECG-gated 3D MRA-MR venography (MRV) technique described in 2019 as "relaxation-enhanced angiography without contrast and triggering (REACT)" may facilitate such evaluation. OBJECTIVE. The purpose of our study was to compare image quality, diagnostic confidence, and interreader agreement between respiratory-triggered REACT and 3D Dixon-based contrast-enhanced MRV (CE-MRV) for evaluating thoracic central venous patency in children and young adults. METHODS. This retrospective study included 42 consecutive children and young adults who underwent MRI of the neck and chest to evaluate central venous patency between August 2019 and January 2021 (median age, 5.2 years; IQR, 1.4-15.1 years; 22 female patients and 20 male patients). Examinations included respiratory-triggered REACT and navigator-gated CE-MRV sequences based on the institution's standard-of-care protocol. Six pediatric radiologists from four different institutions independently reviewed REACT and CE-MRV sequences; they assessed overall image quality (scale, 1-5; 5 = excellent), diagnostic confidence (scale, 1-5; 5 = extremely confident), and presence of clinically relevant artifact(s). Readers classified seven major central vessels as normal or abnormal (e.g., narrowing, thrombosis, or occlusion). Analysis used Wilcoxon signed rank and McNemar tests and Fleiss kappa coefficients. RESULTS. The distribution of overall image quality scores was higher ( p = .02) for REACT than for CE-MRV for one reader (both sequences: median score, 5). Image quality scores were not significantly different between the sequences for the remaining five readers (all p > .05). Diagnostic confidence scores and frequency of clinically relevant artifact(s) were not significantly different between sequences for any reader (all p > .05). Interreader agreement for vessel classification as normal or abnormal was similar between sequences for all seven vessels (REACT: κ = 0.37-0.81; CE-MRV: κ = 0.34-0.81). Pooling readers and vessels, 65.4% of vessels were normal by both sequences; 18.7%, abnormal by both sequences; 9.8%, abnormal by REACT only; and 6.1%, abnormal by CE-MRV only. CONCLUSION. Respiratory-triggered REACT, in comparison with CE-MRV, showed no significant difference in image quality (aside from for one of six readers), diagnostic confidence, or frequency of artifact(s), with similar interreader agreement for vessel classification as normal or abnormal. CLINICAL IMPACT. High-resolution 3D MRV performed without IV contrast material can be used to assess central venous patency in children and young adults.

Background: This study examines whether heterogeneous (HTG) pattern on liver ultrasound (US) identifies children at risk for advanced cystic fibrosis liver disease (aCFLD)., Methods: Prospective 6-year multicenter case-controlled cohort study. Children with pancreatic insufficient cystic fibrosis (CF) aged 3-12 years without known cirrhosis underwent screening US. Participants with HTG were matched (by age, Pseudomonas infection status and center) 1:2 with participants with normal (NL) US pattern. Clinical status and laboratory data were obtained annually and US bi-annually for 6 years. Primary endpoint was development of nodular (NOD) US pattern consistent with aCFLD., Results: 722 participants underwent screening US, with 65 HTG and 592 NL. Final cohort included 55 HTG and 116 NL with ≥ 1 follow-up US. ALT, AST, GGTP, FIB-4, GPR and APRI were higher, and platelets were lower in HTG compared to NL. HTG had a 9.5-fold increased incidence (95% confidence interval [CI]:3.4, 26.7, p<0.0001, 32.7% vs 3.4%) of NOD versus NL. HTG had a sensitivity of 82% and specificity of 75% for subsequent NOD. Negative predictive value of a NL US for subsequent NOD was 96%. Multivariate logistic prediction model that included baseline US, age, and log(GPR) improved the C-index to 0.90 compared to only baseline US (C-index 0.78). Based on survival analysis, 50% of HTG develop NOD after 8 years., Conclusions: Research US finding of HTG identifies children with CF with a 30-50% risk for aCFLD. A score based on US pattern, age and GPR may refine the identification of individuals at high risk for aCFLD., Clinical Trial Registration: Prospective Study of Ultrasound to Predict Hepatic Cirrhosis in CF: NCT 01,144,507 (observational study, no consort checklist)., Competing Interests: Declaration of Competing Interest The authors disclosure the following Sarah Jane Schwarzenberg serves as a: consultant for AbbVie, Michael R Narkewicz serves as a consultant for Vertex, and has received research grants from Gilead, AbbVie and has a family member with stock in Merck. Jean Molleston has research funding from Abbvie, Albireo, Gillead, Shire. Daniel H. Leung has served as a consultant for Merck, Gilead and Vertex and has received research grants from Gilead, Abbvie and Mirum. A. Jay Freeman has done consulting work for AbbVie and Takeda and has received research support from Allergan and Travere Therapeutics. Wikrom Karnsakul has received grants from Albireo Pharma, Gilead, and Travere Therapeutics. Alexander J Towbin received author royalites from Elsevier, served as a consultant to Applied Radiology and received grant funding from the Cystic Fibrosis Foundation. Simon Ling has received research grants from Abbvie and Gilead. The remaining authors disclose no conflicts., (Copyright © 2023 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.)

Background: Survival for children with metastatic hepatoblastoma (HB) remains suboptimal. We report the response rate and outcome of two courses of vincristine/irinotecan/temsirolimus (VIT) in children with high-risk (HR)/metastatic HB., Procedures: Patients with newly diagnosed HB received HR window chemotherapy if they had metastatic disease or a serum alpha-fetoprotein (AFP) level less than 100 ng/mL. Patients received vincristine (days 1 and 8), irinotecan (days 1-5), and temsirolimus (days 1 and 8). Cycles were repeated every 21 days. Responders had either a 30% decrease using RECIST (Response Evaluation Criteria in Solid Tumors) criteria OR a 90% (>1 log 10 decline) AFP decline after two cycles. Responders received two additional cycles of VIT intermixed with six cycles of cisplatin/doxorubicin/5-fluorouracil/vincristine (C5VD). Nonresponders received six cycles of C5VD alone., Results: Thirty-six eligible patients enrolled on study. The median age at enrollment was 27 months (range: 7-170). Seventeen of 36 patients were responders (RECIST and AFP = 3, RECIST only = 4, AFP only = 10). The median AFP at diagnosis was 222,648 ng/mL and the median AFP following two VIT cycles was 19,262 ng/mL. Three-year event-free survival was 47% (95% confidence interval [CI]: 30%-62%), while overall survival was 67% (95% CI: 49%-80%)., Conclusion: VIT did not achieve the study efficacy endpoint. Temsirolimus does not improve the response rate seen in patients treated with vincristine and irinotecan (VI) alone as part of the initial treatment regimen explored in this study. Additionally, AFP response may be a more sensitive predictor of disease response than RECIST in HB., (© 2023 Wiley Periodicals LLC.)

Adrenal tumors other than neuroblastoma are uncommon in children. The most frequently encountered are adrenocortical carcinoma and pheochromocytoma. This paper offers consensus recommendations for imaging of pediatric patients with a known or suspected primary adrenal malignancy other than neuroblastoma at diagnosis and during follow-up., (© 2022 Wiley Periodicals LLC.)

Primary intratesticular tumors are uncommon in children, but incidence and risk of malignancy both sharply increase during adolescence. Ultrasound is the mainstay for imaging the primary lesion, and cross-sectional modalities are often required for evaluation of regional or distant disease. However, variations to this approach are dictated by additional clinical and imaging nuances. This paper offers consensus recommendations for imaging of pediatric patients with a known or suspected primary testicular malignancy at diagnosis and during follow-up., (© 2022 Wiley Periodicals LLC.)

Primary hepatic malignancies are relatively rare in the pediatric population, accounting for approximately 1%-2% of all pediatric tumors. Hepatoblastoma and hepatocellular carcinoma are the most common primary liver malignancies in children under the age of 5 years and over the age of 10 years, respectively. This paper provides consensus-based imaging recommendations for evaluation of patients with primary hepatic malignancies at diagnosis and follow-up during and after therapy., (© 2022 Wiley Periodicals LLC.)

Ovarian tumors in children are uncommon. Like those arising in the adult population, they may be broadly divided into germ cell, sex cord, and surface epithelium subtypes; however, germ cell tumors comprise the majority of lesions in children, whereas tumors of surface epithelial origin predominate in adults. Diagnostic workup, including the use of imaging, requires an approach that often differs from that required in an adult. This paper offers consensus recommendations for imaging of pediatric patients with a known or suspected primary ovarian malignancy at diagnosis and during follow-up., (© 2022 Wiley Periodicals LLC.)

The most common pediatric extragonadal pelvic cancers include germ cell tumors, sacrococcygeal teratomas, and rhabdomyosarcomas (arising from the urinary bladder, prostate, paratesticular tissues, vagina, uterus, and perineum). This paper describes the radiological and nuclear medicine features of these entities and provides consensus-based recommendations for the assessment at diagnosis, during, and after treatment., (© 2022 Wiley Periodicals LLC.)

Right lower quadrant (RLQ) pain is a common clinical presentation in children, and accurate clinical diagnosis remains challenging given that this nonspecific presentation is associated with numerous surgical and nonsurgical conditions. The broad differential diagnosis varies by patient age and sex. Important considerations in the selection of a diagnostic imaging strategy include the sequencing, performance, and cost of tests. This article provides a comprehensive narrative review of the diagnostic imaging of RLQ pain in children and adolescents, including a discussion of the complementary roles of ultrasound, CT, and MRI; description of key imaging findings based on available evidence; and presentation of salient differential diagnoses. Subspecialized pediatric emergency medicine and surgical perspectives are also provided as further clinical insight into this common, but often challenging, scenario. Finally, the current status of imaging of RLQ pain in children and adolescents is summarized on the basis of expert consensus.

Point-of-care ultrasound (POCUS) is widely used for both diagnostic and therapeutic purposes. With its many advantages, including ease of use, real-time multisystem assessment, affordability, availability, and accuracy, it has been adopted by all medical specialties. Despite its advantages, the lack of standard workflow and automated billing solutions makes it difficult to launch a comprehensive POCUS program. In this work, we describe how we created and implemented an efficient standardized EHR-based workflow for POCUS that has been used across multiple division and settings within our organization., (© 2022. The Author(s) under exclusive licence to Society for Imaging Informatics in Medicine.)

As the immuno-oncology field continues the rapid growth witnessed over the past decade, optimising patient outcomes requires an evolution in the current response-assessment guidelines for phase 2 and 3 immunotherapy clinical trials and clinical care. Additionally, investigational tools-including image analysis of standard-of-care scans (such as CT, magnetic resonance, and PET) with analytics, such as radiomics, functional magnetic resonance agents, and novel molecular-imaging PET agents-offer promising advancements for assessment of immunotherapy. To document current challenges and opportunities and identify next steps in immunotherapy diagnostic imaging, the National Cancer Institute Clinical Imaging Steering Committee convened a meeting with diverse representation among imaging experts and oncologists to generate a comprehensive review of the state of the field., Competing Interests: Declaration of interests EGEdV reports institutional financial support for clinical trials or contracted research from Amgen, Genentech, Roche, CytomX, G1 Therapeutics, Bayer, Synthon, Servier, Regeneron, Crescendo Biologics, GE Healthcare, and AstraZeneca; institutional financial support for advisory boards and consultancy from National Surgical Adjuvant Breast and Bowel Project, Daiichi Sankyo, and Crescendo Biologics; and non-financial interests, including non-renumerated activities and public positions as a member of the European Society for Medical Oncology (ESMO)–Magnitude of Clinical Benefit Scale working party, chair of the ESMO Cancer Medicines Working Group, co-chair of the RECIST committee, and a member of the expert panel for the selection of WHO Essential Medicine List. MDF reports institutional grant or contract payments from Merck, Bristol Myers Squibb, ImaginAb, and Carisma Therapeutics, and consulting fees and meeting and travel support from ImaginAb. DL reports stock ownership in Bristol Myers Squibb. UM reports grants, royalties and license, consulting fees, meeting and travel support, licensed patents, leadership interest, stock, receipt of materials, and other financial or non-financial interests in CytoSite Biopharma. TYP reports grants or contracts from the Pediatric Brain Tumor Consortium Neuroimaging Center; royalties from Springer; and honoraria for grand rounds, visiting professorships, and invited lectures from Cornell University, University of California San Francisco, and University of Rochester. DP reports grants to or contracts with his institution from Siemens, 511 Pharma, Nordic nanovector, Lantheus, and Fusion Pharmaceuticals; royalties or licenses to him and his institution from Trevarx; consulting fees from Siemens, Fusion Pharmaceuticals, 511 Pharma, Ipsen, Bayer, Molecular Targeting Technologies, and Curium; patents from At-211 parthanatrace; participation on an Isotope Technologies Munich (ITM) data and safety monitoring committee; and stock with Molecular Targeting Technologies and Trevarx. LS reports institutional grants or contracts from Johnson & Johnson, patents from Varian Medial Systems, and data and safety monitoring board or advisory board participation with Merck, BMS, and Regeneron. YT provided medical writing assistance funded by NCI. AJT reports an institutional research grant from the Cystic Fibrosis Foundation, royalties from Elsevier, consulting fees from Applied Radiology, travel support from KLAS Research, and unpaid leadership roles in the Society for Imaging Informatics in Medicine and the Society for Pediatric Radiology. RLW reports grants or contracts from Bristol Myers Squibb, Bayer, Actinium Pharmaceuticals, ITM, Siemens, and NIH; consulting fees from Seno medical, Actinium Pharmaceuticals, and Clarity Pharmaceuticals; payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing, or educational events from ITM, Voximetry, American College of Nuclear Medicine, and NIH (not for this workshop or manuscript); support for meetings and travel from the Society of Nuclear Medicine and the World Theranostics Conference; data and safety monitoring board and advisory board participation from Bayer; a recent past-presidency at Society of Nuclear Medicine and Molecular Imaging; and stock in Clarity Pharmaceuticals and Voximetry. JW reports grants from Bristol Myers Squibb and Sephora; institutional royalties for xenogeneic DNA vaccines, Newcastle disease viruses for cancer therapy, myeloid-derived suppressor cell assay, and prediction of responsiveness to treatment with immunomodulatory therapeutics and method of monitoring abscopal effects during such treatment; licenses for anti-PD1 antibodies, anti-CTLA4 antibodies, and anti-GITR antibodies, and methods of use thereof; consulting fees from Amgen, Apricity, Ascentage Pharma, Astellas, AstraZeneca, Bicara Therapeutics, Boehringer Ingelheim, Bristol Myers Squibb, CellCarta, Chugai, Daiichi Sankyo, Dragonfly, Georgiamune, Idera, Imvaq, Larkspur, Maverick Therapeutics, Merck, Psioxus, Recepta, Trishula, Sellas, Surface Oncology, and Werewolf therapeutics; support for meetings and travel from Society for Immunotherapy of Cancer (SITC) and Parker Institute for Cancer Immunotherapy; a leadership or fiduciary role in SITC; and stock in Apricity, CellCarta, Ascentage, Beigene, Imvaq, Linneaus, Georgiamune, Maverick, Tizona Pharma, and Trieza. JW also reports patents for recombinant poxviruses for cancer immunotherapy, phosphatidylserine targeting agents, immunosuppressive follicular helper-like T cells modulated by immune checkpoint blockade, identifying and treating subjects at risk for checkpoint blockade therapy-associated colitis, CAR(+) T cells targeting differentiation antigens as means to treat cancer, anti-CD40 agonist monoclonal antibody fused to monophosphoryl lipid A for cancer therapy, and engineered vaccinia viruses for cancer immunotherapy. AMW reports consulting fees, a leadership or fiduciary role (board member), and stock options in ImaginAb; and speaker honoraria payments from Roche and AstraZeneca. All other authors declare no competing interests., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

Background: Nodular liver (NOD) in cystic fibrosis (CF) suggests advanced CF liver disease (aCFLD); little is known about progression of liver disease (LD) after detection of sonographic NOD., Methods: Clinical, laboratory, and ultrasound (US) data from Prediction by Ultrasound of the Risk of Hepatic Cirrhosis in CFLD Study participants with NOD at screening or follow-up were compared with normal (NL). Linear mixed effects models were used for risk factors for LD progression and Kaplan-Meier estimator for time-to-event., Results: 54 children with NOD (22 screening, 32 follow-up) and 112 NL were evaluated. Baseline (BL) and trajectory of forced expiratory volume, forced vital capacity, height/BMI z-scores were similar in NOD vs NL. Platelets were lower in NOD at BL (250 vs 331×10 3 /microL; p < 0.001) and decreased by 8600/year vs 2500 in NL. Mean AST to Platelet Ratio Index (1.1 vs 0.4; p < 0.001), Fibrosis-4 Index (0.4 vs 0.2, p < 0.001), and spleen size z-score (SSZ) [1.5 vs 0.02; p < 0.001] were higher in NOD at BL; SSZ increased by 0.5 unit/year in NOD vs 0.1 unit/year in NL. Median liver stiffness (LSM) by transient elastography was higher in NOD (8.2 kPa, IQR 6-11.8) vs NL (5.3, 4.2-7, p < 0.0001). Over 6.3 years follow-up (1.3-10.3), 6 NOD had esophageal varices (cumulative incidence in 10 years: 20%; 95% CI: 0.0%, 40.0%), 2 had variceal bleeding, and 2 underwent liver transplantation; none had ascites or hepatic encephalopathy. No NL experienced liver-related events., Conclusions: NOD developed clinically evident portal hypertension faster than NL without worse growth or lung disease., (Copyright © 2022. Published by Elsevier B.V.)

A differential diagnosis based on a patient's age, clinical presentation, and serum α-fetoprotein level will help guide the initial imaging workup in children with a liver lesion. Children vary significantly in size, the ability to stay still, and the ability to breath hold for imaging examinations. Choosing and tailoring imaging techniques and protocols for each indication and age group is important for optimal care with minimal invasiveness. The need for sedation or anesthesia can be obviated by using techniques like feed and bundle, distraction, contrast-enhanced US, and motion-insensitive sequences for MRI. US is often the first imaging modality used in children with a suspected abdominal mass. Once a hepatic lesion is confirmed, multiphasic contrast-enhanced MRI is recommended for most lesions as the next imaging modality allowing full characterization of the lesion and assessment of the liver parenchyma. Contrast-enhanced CT can also be performed for assessment of pediatric focal liver lesions, especially in patients who have a contraindication to MRI. Contrast-enhanced US has shown promise to decrease the need for MRI or CT in some lesions such as hemangioma and focal nodular hyperplasia. Children with a history of malignancy can develop multiple types of hepatic lesions at various stages, including infections during an immunocompromised state, manifesting as focal liver lesions. Based on available limited data in the literature and the collective experiences of the Liver Imaging and Reporting Data System Pediatric Working Group, the authors provide guidelines for the imaging workup of pediatric focal liver lesions with an indication- and age-based approach and discuss the selection and performance of various imaging techniques and modalities. © RSNA, 2022 See the invited commentary by Chojniak and Boaventura in this issue.

BACKGROUND. The simplified MR index of activity (MaRIA) score is used to assess the severity of small-bowel inflammation without use of IV contrast material. OBJECTIVE. The purposes of this study were to assess interreader agreement on the use of simplified MaRIA scores for evaluation of the inflammatory activity of terminal ileal Crohn disease in children and young adults and to assess whether simplified MaRIA scores change after biologic medical therapy. METHODS. This analysis was ancillary to a previously reported primary prospective research investigation. The study included 20 children and young adults with newly diagnosed ileal Crohn disease and 15 healthy control participants who underwent research small-bowel MRI examinations between December 2018 and October 2021. The participants with Crohn disease underwent baseline MRI and MRI 6 weeks and 6 months after beginning anti-tumor necrosis factor α-treatment as well as weighted pediatric Crohn disease activity index (wPCDAI) and C-reactive protein (CRP) assessment on the day of each examination. Control participants underwent one MRI examination. Four pediatric radiologists independently assigned simplified MaRIA scores using axial and coronal T2-weighted SSFSE images. Median simplified MaRIA score among readers was computed. Interreader agreement was assessed with Fleiss kappa coefficients and intra-class correlation coefficient (ICC). Analysis included the Mann-Whitney U test, Friedman test, and Spearman rank correlation. RESULTS. Simplified MaRIA scores (across time points and study groups) had substantial interreader agreement (κ = 0.65 [95% CI, 0.56-0.74]; ICC, 0.71 [95% CI, 0.63-0.78]). Median scores were higher in participants with Crohn disease at baseline than in healthy control participants (3.5 [IQR, 2.5-4.9] vs 0.5 [IQR, 0-2.0]; p < .001). Scores decreased after medical treatment in participants with Crohn disease ( p = .005). The median score was 3.5 (IQR, 2.5-4.9) at baseline, 2.3 (IQR, 1.6-3.9) at 6 weeks, and 2.0 (IQR, 0.5-2.5) at 6 months. In participants with Crohn disease, median scores had significant correlations with wPCDAI (ρ = 0.46 [95% CI, 0.18-0.64]; p < .001) and CRP level (ρ = 0.48 [95% CI, 0.27-0.65]; p < .001). CONCLUSION. Radiologists had substantial agreement in use of simplified MaRIA scores to assess intestinal inflammation in ileal Crohn disease. Scores changed over time after medical therapy. CLINICAL IMPACT. The results support the simplified MaRIA score as an objective MRI-based clinical measure of intestinal inflammation in children and young adults with Crohn disease.

Objectives: Cystic fibrosis liver disease (CFLD) begins early in life. Symptoms may be vague, mild, or nonexistent. Progressive liver injury may be associated with decrements in patient health before liver disease is clinically apparent. We examined Health-Related Quality of Life (HRQOL) in children enrolled in a multi-center study of CFLD to determine the impact of early CFLD on general and disease-specific QOL., Methods: Ultrasound (US) patterns of normal (NL), heterogeneous (HTG), homogeneous (HMG), or nodular (NOD) were assigned in a prospective manner to predict those at risk for advanced CFLD. Parents were informed of results. We assessed parent/child-reported (age ≥5 years) HRQOL by PedsQL 4.0 Generic Core and CF Questionnaire-revised (CFQ-R) prior to US and annually. HRQOL scores were compared by US pattern at baseline (prior to US), between baseline and 1 year and at 5 years. Multivariate analysis of variance (MANOVA) with Hotelling-Lawley trace tested for differences among US groups., Results: Prior to US, among 515 participants and their parents there was no evidence that HTG or NOD US was associated with reduced PedsQL/CFQ-R at baseline. Parents of NOD reported no change in PedsQL/CFQ-R over the next year. Child-report PedsQL/CFQ-R (95 NL, 20 NOD) showed improvement between baseline and year 5 for many scales, including Physical Function. Parents of HMG children reported improved CFQ-R scores related to weight., Conclusions: Early undiagnosed or pre-symptomatic liver disease had no impact on generic or disease-specific HRQoL, and HRQoL was remarkably stable in children with CF regardless of liver involvement., Competing Interests: S.J.S. serves as a consultant for Nestle, UpToDate, and AbbVie. A.J.F. has grant/research support through Travere Therapeutics and Allergan; serves as an advisor for Abbvie and Takeda. W.K. has received research grants from Gilead Sciences and Albireo Pharma; serves as an advisor for Mirum and Travere Therapeutics. D.H.L. has grant/research support from Abbvie, Gilead, and Mirum; serves as a consultant for Gilead, Vertex, and Merck. S.C.L. receives research funding from Abbvie and Gilead and serves as a consultant for Abbvie JPM has research funding from Gillead, Abbvie, Albireo, Mirum. K.F.M. is a consultant for Albireo and Gilead. M.R.N. serves as a consultant for Vertex, has received research grants from Gilead, AbbVie, and has a family member with stock in Merck. The remaining authors report no conflicts of interest., (Copyright © 2022 by European Society for European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Background: Hepatoblastoma (HB) requires surgical resection for cure, but only 20-30% of patients have resectable disease at diagnosis. Patients who undergo partial hepatectomy at diagnosis have historically received 4-6 cycles of adjuvant chemotherapy; however, those with 100% well-differentiated fetal histology (WDF) have been observed to have excellent outcomes when treated with surgery alone., Patients and Methods: Patients on the Children's Oncology Group non randomized, multicenter phase III study, AHEP0731, were stratified based on Evan's stage, tumor histology, and serum alpha-fetoprotein level at diagnosis. Patients were eligible for the very low risk stratum of surgery and observation if they had a complete resection at diagnosis and rapid central histologic review demonstrated HB with 100% WDF histology., Results: A total of 8 eligible patients were enrolled on study between September 14, 2009 and May 28, 2014. Outcome current to 06/30/2020 was used in this analysis. The median age at enrollment was 22.5 months (range: 8-84 months) and the median AFP at enrollment was 714 ng/ml (range: 18-77,747 ng/mL). With a median follow-up of 6.6 years (range: 3.6-9.8 years), the 5-year event-free (EFS) and overall survival (OS) were both 100%., Conclusion: This report supports that HB with 100% WDF histology completely resected at diagnosis is curable with surgery only. The development of evidence-based surgical guidelines utilizing criteria based on PRETEXT group, vascular involvement (annotation factors), tumor-specific histology and corresponding biology will be crucial for optimizing which patients are candidates for resection at diagnosis followed by observation., Level of Evidence: Prognosis study, Level I evidence., (Copyright © 2022. Published by Elsevier Inc.)

BACKGROUND. Pediatric hepatocellular carcinoma (HCC) is an aggressive malignancy for which imaging findings remain poorly described. In comparison with adult HCC, pediatric HCC more commonly occurs without underlying risk factors, and standardized surveillance guidelines for those with predispositions are lacking. OBJECTIVE. The purpose of this article was to evaluate imaging findings of nonfibrolamellar pediatric HCC and to identify associations between these imaging findings and the presence of predisposing factors. METHODS. This retrospective study included children (≤ 18 years) with histologically confirmed nonfibrolamellar HCC who underwent multiphase CT or MRI at one of four academic children's hospitals between July 2009 and April 2019. Surveillance regimens in children with predispositions were at the discretion of treating physicians. Clinical characteristics were recorded. Scan indications were classified as surveillance versus clinical signs and symptoms. Images from all sites were submitted to a cloud-based server. Two radiologists independently assessed imaging features of HCC, including tumor size, tumor in vein, Pre-Treatment Extent of Tumor (PRETEXT) stage, and LI-RADS major features of adult HCC. Imaging findings were compared between patients with and without predispositions. RESULTS. The study included 39 patients: 17 with predispositions (mean age, 10.5 ± 4.5 years; nine boys, eight girls) and 22 without predispositions (mean age, 11.3 ± 5.1 years; 12 boys, 10 girls). Scan indication was surveillance in 14/17 patients with predispositions versus 0/22 patients without predispositions ( p < .001). Patients with versus those without predispositions had smaller tumor size (reader 1: 6.0 vs 11.9 cm [ p = .003]; reader 2: 6.0 vs 12.9 cm [ p < .001]) and less frequent tumor in vein (reader 1: 0% vs 41% [ p = .002]; reader 2: 0% vs 36% [ p = .006]). PRETEXT stage IV disease was observed in 18% (both readers) of patients with predispositions versus 50-55% of patients without predispositions. No LI-RADS major feature of adult HCC showed a significant difference in frequency between patients with and without predispositions for either reader (all p > .05). CONCLUSION. Among children with HCC, those with predispositions exhibited smaller and lower-stage tumors and less frequent tumor in vein, likely because of surveillance imaging. CLINICAL IMPACT. The study supports the role of routine surveillance imaging in children with HCC predispositions to facilitate earlier detection. Standardization of surveillance guidelines remains needed.

BACKGROUND. Changes in intestinal motility in patients with newly diagnosed Crohn disease have historically been evaluated primarily in a subjective manner. OBJECTIVE. The purpose of this study was to assess longitudinal changes in objective intestinal motility scores in children and young adults with newly diagnosed ileal Crohn disease treated with biologic (anti-tumor necrosis factor-α) medical therapy compared with those in control participants. METHODS. This prospective study included 20 children and young adults (eight female and 12 male patients; mean age, 14.6 ± 2.1 [SD] years) with newly diagnosed ileal Crohn disease who were recruited between December 2018 and October 2021 as well as 15 control participants without any known gastrointestinal conditions (eight female and seven male patients; mean age, 18.1 ± 4.4 years). All participants underwent research MRI examinations of the small bowel, including dynamic cine 2D SSFP sequences. Patients with Crohn disease underwent additional research MRI examinations performed at both 6 weeks and 6 months after initiation of biologic therapy. Two operators independently derived terminal ileal intestinal motility scores from the dynamic cine sequences by use of FDA-approved software (with higher scores indicating greater intestinal motility). Intestinal motility scores were compared between patient and control groups by use of t tests, whereas changes in intestinal motility scores after treatment were assessed using linear mixed models. Interoperator absolute agreement was assessed using the intra-class correlation coefficient (ICC). RESULTS. Mean terminal ileal intestinal motility scores were not significantly different between patients with newly diagnosed ileal Crohn disease and control participants (for operator 1, 180.9 ± 63.3 vs 229.7 ± 115.2, respectively [ p = .12]; for operator 2, 175.0 ± 62.2 vs 236.4 ± 117.4, respectively [ p = .05]). Mean intestinal motility scores changed over time compared with baseline in response to biologic therapy, for operator 1 (180.9 ± 63.3 at baseline, 248.1 ± 104.9 at 6 weeks after treatment initiation, and 249.1 ± 73.2 at 6 months after treatment initiation [ p = .04]) and operator 2 (175.0 ± 62.2 at baseline, 247.8 ± 112.7 at 6 weeks after treatment initiation, and 239.6 ± 72.7 at 6 months after treatment initiation [ p = .03]). Absolute agreement in intestinal motility scores was excellent between operators (ICC = 0.89). CONCLUSION. MRI measurements of intestinal motility are dynamic in children and adults with newly diagnosed small-bowel Crohn disease, showing early increases in response to biologic therapy. CLINICAL IMPACT. MRI-based intestinal motility scores may aid individualized assessment of disease activity and treatment response in patients with small-bowel Crohn disease.

Transferring medical imaging studies from one institution to another is a common occurrence in today's medical practice. For the past two decades, radiology departments have relied on physical media (compact discs and digital video disks) and human couriers to accomplish image transfer. This mode of transfer is slow, prone to failure, and reliant on outdated technology. To address these shortcomings, multiple image-sharing vendors have created electronic, cloud-based solutions. While these solutions solve multiple problems, a new problem has been introduced: it is difficult to send or receive images across image-sharing platforms. In this work, we describe how we have developed a solution to share images across multiple vendor platforms., (© 2022. The Author(s) under exclusive licence to Society for Imaging Informatics in Medicine.)

BACKGROUND. Intestinal inflammation is associated with radiologic and histologic hyperemia. A paucity of studies have used MRI to measure mesenteric blood flow in patients with Crohn disease. OBJECTIVE. The purpose of this study was to evaluate the application of velocity-encoded phase-contrast MRI for measuring mesenteric blood flow in patients with newly diagnosed small-bowel Crohn disease. METHODS. This prospective study included 20 patients with ileal Crohn disease newly diagnosed between December 2018 and October 2021 (eight female participants, 12 male participants; median age, 14.0 years), and 15 healthy control participants (eight female participants, seven male participants; median age, 17.0 years). Patients with Crohn disease underwent investigational MRI and laboratory assessments at diagnosis and at 6 weeks and 6 months after initiating anti-tumor necrosis factor-α medical therapy; control participants underwent a single investigational MRI examination. All MRI examinations included a velocity-encoded phase-contrast acquisition, which was used to measure blood flow in the abdominal aorta, superior mesenteric artery (SMA), and superior mesenteric vein (SMV). Mann-Whitney U test was used to compare blood flow measurements (ratios of SMA and SMV blood flow to aorta blood flow [hereafter, SMA-to-aorta and SMV-to-aorta blood flow, respectively]) between groups; Friedman test was used to evaluate temporal changes in blood flow. Spearman correlation was used to assess relationships between blood flow measurements and laboratory markers of intestinal inflammation. Diagnostic performance was assessed by ROC analysis. RESULTS. At baseline, SMA-to-aorta blood flow in patients versus control participants was 0.44 versus 0.30 ( p = .003), and SMV-to-aorta blood flow was 0.36 versus 0.21 ( p = .002). At 6 weeks and 6 months, SMA-to-aorta blood flow in patients decreased to 0.30 and 0.27 ( p < .001), and SMV-to-aorta blood flow decreased to 0.27 and 0.21 ( p = .02), respectively. SMA-to-aorta and SMV-to-aorta blood flow were positively correlated with C-reactive protein (rho, 0.34 [ p = .01] and 0.35 [ p = .008], respectively) and fecal calprotectin (rho, 0.34 [ p = .01] vs 0.47 [ p < .001]). AUCs for differentiating patients from controls were 0.79 for SMA-to-aorta (sensitivity, 60%; specificity, 100%) and 0.82 for SMV-to-aorta (sensitivity, 75%; specificity, 87%) blood flow. CONCLUSION. Mesenteric blood flow is quantifiable using velocity-encoded phase-contrast MRI. The measurements differ between patients with ileal Crohn disease and healthy control participants and change in response to medical therapy. CLINICAL IMPACT. MRI-based mesenteric blood flow measurements provide a potential novel marker of intestinal inflammation.

Photodocumentation is a subset of visible light imaging and is an important growing segment of enterprise imaging. Medical videography is another subset of visible light imaging that shares many of the challenges of photodocumentation. Medical photographs are used to document clinical conditions, support diagnosis, guide, and document procedures and to enable collaboration among colleagues. They also play a significant role in patient engagement and are a mechanism for patients to share information with their provider without the need for a clinical office visit. The content of medical photographs raises issues for acquisition, management, storage, and access. Medical photographs may contain protected health information, and these images benefit from the standardized, secure processes inherent in any enterprise imaging program. The ability to securely acquire images on mobile, and sometimes personally owned devices, is a necessity. In addition to containing protected health information, photograph content can be sensitive or gruesome or the images may be used for forensic purposes. These types of images require additional protections. Access to these images should be role-based and auditable. To properly identify photographs and to convey information about their acquisition parameters new metadata requirements and mechanisms for its association with the imaging files are evolving. Institutional policies need to be developed to define the organization's requirements for medical photography, including consent processes. Existing policies such as those defining the designated record set and legal health record should address the management of medical photography., (© 2022. The Author(s).)

In today's workflow, radiologists rely on the picture archiving and communication system (PACS) to view images. Unplanned downtime causes significant delays in patient care and lengthy downtimes can have lasting effects on patient care and end-user confidence. The purpose of this manuscript is to describe the planning and implementation of a disaster drill where the departmental PACS was taken off-line and the hospital enterprise viewer was used for departmental image interpretation., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Tuberous sclerosis complex (TSC) results in neurodevelopmental phenotypes, benign tumors, and cysts throughout the body. Recent studies show numerous rare findings in TSC. Guidelines suggest routine abdominal and chest imaging to monitor these thoracoabdominal findings, but imaging is not uniformly done across centers. Thus, the prevalence of many findings is unknown. To answer this, we categorized the clinical reads of 1398 thoracoabdominal scans from 649 patients of all ages in the Cincinnati Children's Hospital TSC Repository Database., Results: Typical TSC findings were present in many patients: kidney cysts (72%), kidney fat-containing angiomyolipomas (51%), kidney lipid-poor angiomyolipomas (27%), liver angiomyolipomas (19%), and lung nodules thought to represent multifocal micronodular pneumocyte hyperplasia (MMPH) (18%). While many features were more common in TSC2 patients, TSC1 patients had a higher prevalence of MMPH than TSC2 patients (24% versus 13%, p = 0.05). Many rare findings (e.g., lymphatic malformations and liver masses) are more common in TSC than in the general population. Additionally, most thoracoabdominal imaging findings increased with age except kidney cysts which decreased, with the 0-10 years age group having the highest percentage (69% 0-10 years, 49% 10-21 years, 48% 21 + years, p < 0.001). Finally, in our population, no patients had renal cell carcinoma found on abdominal imaging., Conclusions: These results show that regular thoracoabdominal scans in TSC may show several findings that should not be ignored or, conversely, over-reacted to when found in patients with TSC. Female sex, TSC2 mutation, and age are risk factors for many thoracoabdominal findings. The data suggest novel interactions of genetic mutation with pulmonary nodules and age with renal cysts. Finally, in agreement with other works, these findings indicate that several rare thoracoabdominal imaging findings occur at higher rates in the TSC population than in the general population. This work supports obtaining detailed thoracoabdominal imaging in patients with TSC., (© 2022. The Author(s).)

Background: The Children's Oncology Group (COG) adopted cisplatin, 5-flourouracil, and vincristine (C5V) as standard therapy after the INT-0098 legacy study showed statistically equivalent survival but less toxicity in comparison with cisplatin and doxorubicin. Subsequent experience demonstrated doxorubicin to be effective in patients with recurrent disease after C5V, and this suggested that it could be incorporated to intensify therapy for patients with advanced disease., Methods: In this nonrandomized, phase 3 COG trial, the primary aim was to explore the feasibility and toxicity of a novel therapeutic cisplatin, 5-flourouracil, vincristine, and doxorubicin (C5VD) regimen with the addition of doxorubicin to C5V for patients considered to be at intermediate risk. Patients were eligible if they had unresectable, nonmetastatic disease. Patients with a complete resection at diagnosis and local pathologic evidence of small cell undifferentiated histology were also eligible for an assessment of feasibility., Results: One hundred two evaluable patients enrolled between September 14, 2009, and March 12, 2012. Delivery of C5VD was feasible and tolerable: the mean percentages of the target doses delivered were 96% (95% CI, 94%-97%) for cisplatin, 96% (95% CI, 94%-97%) for 5-fluorouracil, 95% (95% CI, 93%-97%) for doxorubicin, and 90% (95% CI, 87%-93%) for vincristine. Toxicity was within expectations, with death as a first event in 1 patient. The most common adverse events were febrile neutropenia (n = 55 [54%]), infection (n = 48 [47%]), mucositis (n = 31 [30%]), hypokalemia (n = 39 [38%]), and elevated aspartate aminotransferase (n = 28 [27%]). The 5-year event-free and overall survival rates for the 93 patients who did not have complete resection at diagnosis were 88% (95% CI, 79%-93%) and 95% (95% CI, 87%-98%), respectively., Conclusions: The addition of doxorubicin to the previous standard regimen of C5V is feasible, tolerable, and efficacious, and this suggests that C5VD is a good regimen for future clinical trials., (© 2021 American Cancer Society.)