ACR Appropriateness Criteria® Urinary Tract Infection-Child: 2023 Update.

Urinary tract infection (UTI) is a frequent infection in childhood. The diagnosis is usually made by history and physical examination and confirmed by urine analysis. Cystitis is infection or inflammation confined to the bladder, whereas pyelonephritis is infection or inflammation of kidneys. Pyelonephritis can cause renal scarring, which is the most severe long-term sequela of UTI and can lead to accelerated nephrosclerosis, leading to hypertension and chronic renal failure. The role of imaging is to guide treatment by identifying patients who are at high risk to develop recurrent UTIs or renal scarring. This document provides initial imaging guidelines for children presenting with first febrile UTI with appropriate response to medical management, atypical or recurrent febrile UTI, and follow-up imaging for children with established vesicoureteral reflux. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision process support the systematic analysis of the medical literature from peer reviewed journals. Established methodology principles such as Grading of Recommendations Assessment, Development, and Evaluation or GRADE are adapted to evaluate the evidence. The RAND/UCLA Appropriateness Method User Manual provides the methodology to determine the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where peer reviewed literature is lacking or equivocal, experts may be the primary evidentiary source available to formulate a recommendation.

ACR Appropriateness Criteria® Soft Tissue Vascular Anomalies: Vascular Malformations and Infantile Vascular Tumors (Non-CNS)-Child.

Soft tissue vascular anomalies may be composed of arterial, venous, and/or lymphatic elements, and diagnosed prenatally or later in childhood or adulthood. They are divided into categories of vascular malformations and vascular tumors. Vascular malformations are further divided into low-flow and fast-flow lesions. A low-flow lesion is most common, with a prevalence of 70%. Vascular tumors may behave in a benign, locally aggressive, borderline, or malignant manner. Infantile hemangioma is a vascular tumor that presents in the neonatal period and then regresses. The presence or multiple skin lesions in an infant can signal underlying visceral vascular anomalies, and complex anomalies may be associated with overgrowth syndromes. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision process support the systematic analysis of the medical literature from peer reviewed journals. Established methodology principles such as Grading of Recommendations Assessment, Development, and Evaluation or GRADE are adapted to evaluate the evidence. The RAND/UCLA Appropriateness Method User Manual provides the methodology to determine the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where peer reviewed literature is lacking or equivocal, experts may be the primary evidentiary source available to formulate a recommendation.

Partial visualization of appendix on ultrasound: What does it mean in the child with suspected appendicitis?

BACKGROUND: Visualization of the entire appendix, including the tip, is thought, but has not been demonstrated, to be important for exclusion of appendicitis by ultrasound. OBJECTIVE: To determine if incomplete visualization of the appendix has negative clinical ramifications including missed appendicitis. METHODS: Under IRB approval we retrospectively reviewed right lower quadrant ultrasound reports from January 2017 to December 2020 to identify examinations with impressions of full visualization of the normal appendix, non-visualization of the appendix with and without secondary findings of appendicitis, and partial visualization of the appendix. Electronic health records were reviewed for follow-up imaging within 48 h, and surgery with pathology reports (if available). RESULTS: 12,193 examinations were included. 4171 (34.2 %) had full visualization of a normal appendix, 5369 (44.0 %) had non-visualization with no secondary findings, and 234 (1.9 %) had non-visualization with secondary findings, The frequencies of appendicitis in these three groups were 34 (0.8 %), 283 (5.3 %), and 127 (54.3 %) respectively. The appendix was partially visualized in 338 (2.8 %) patients with secondary findings present in 53 (15.6 %). Partial visualization without secondary findings had a similar frequency (4.9 %, 14/285) of appendicitis to non-visualized appendix without secondary findings (p = 0.797) and a higher frequency than full visualization of a normal appendix (p < 0.0001). Partial visualization with secondary findings had similar rates (54.7 %, 29/53) to non-visualized appendix with secondary findings (p = 0.953). CONCLUSION: Partial visualization of the appendix with ultrasound (with and without secondary findings) is associated with similar frequencies of appendicitis as non-visualization of appendix (with and without secondary findings).

Association Between MR Elastography Liver Stiffness and Histologic Liver Fibrosis in Children and Young Adults With Autoimmune Liver Disease.

Background: Liver fibrosis is an important clinical endpoint of progression of autoimmune liver disease (AILD); its monitoring would benefit from noninvasive imaging tools. Objective: To assess the relationship between MR elastography (MRE) liver stiffness measurements and histologic liver fibrosis, as well as to evaluate the performance of MRE and biochemical-based clinical markers for stratifying histologic liver fibrosis severity, in children and young adults with AILD. Methods: This retrospective study used an existing institutional registry of children and young adults diagnosed with AILD [primary sclerosing cholangitis (PSC), ASC (autoimmune sclerosing cholangitis), or AIH (autoimmune hepatitis)]. The registry was searched to identify patients who underwent both a research abdominal 1.5-T MRI that included liver MRE (performed for registry enrollment) and a clinically indicated liver biopsy within a 6-month interval. MRE used a 2D gradient-recalled echo sequence. One analyst measured mean liver shear stiffness (kPa) for each examination. Laboratory markers of liver fibrosis [aspartate aminotransferase-to-platelet ratio index (APRI) and fibrosis-4 score (FIB-4)] were recorded. For investigational purposes, one pathologist determined histologic METAVIR liver fibrosis stage, blinded to clinical and MRI data. Spearman rank-order correlation was calculated between MRE liver stiffness and METAVIR liver fibrosis stage. ROC analysis was used to evaluate diagnostic performance for identifying advanced fibrosis (i.e., differentiating METAVIR F0-F1 from F2-F4 fibrosis), calculating sensitivity and specificity at the Youden's index. Results: The study included 46 patients (median [IQR] age, 16.6 [13.7-17.8] years; 20 female, 26 male); 12 had PSC, 10 had ASC, and 24 had AIH. Median MRE liver stiffness was 2.9 kPa (IQR: 2.2-4.0 kPa). MRE liver stiffness and METAVIR fibrosis stage showed strong positive correlation (rho=0.68). For identifying advanced liver fibrosis, MRE liver stiffness had AUC of 0.81, with sensitivity of 65.4% and specificity of 90.0%; APRI had AUC of 0.72, with sensitivity of 60.0% and specificity of 85.0%; and FIB-4 had AUC of 0.71, with sensitivity of 64.0% and 85.0%. Conclusion: MRE liver stiffness measurements were associated with histologic liver fibrosis severity. Clinical Impact: The findings support a role for MRE in noninvasive monitoring of liver stiffness, a surrogate for fibrosis, in children and young adults with AILD.

Multifocal Insulinoma as the Unique Presenting Feature of MEN1 in an Adolescent.

Introduction Multiple Endocrine Neoplasia Type 1 (MEN1) is an autosomal dominant inherited disorder defined by the presence of two of the following endocrinopathies: primary hyperparathyroidism, anterior pituitary tumors, and duodenopancreatic neuroendocrine tumors (NETs). NETs, which can secrete hormones including insulin, gastrin, and glucagon, among others, are common in patients with MEN1 and are a major cause of morbidity and premature death. NETs are more common later in life, with very few cases described in children. Here, we describe a unique case of an adolescent with multifocal pancreatic NETs as the single presenting feature of MEN1. Case Presentation A 13-year-old healthy male presented with severe weakness, altered mental status, and syncope in the setting of a venous blood glucose (BG) of 36 mg/dL. Workup showed an elevated insulin level (14 mcIU/mL) when BG was 39 mg/dL with positive response to glucagon, concerning for hyperinsulinism. Diazoxide and chlorothiazide were started but not well tolerated secondary to emesis. Three suspected NETs were identified by MRI and 68-Ga DOTATATE PET-CT imaging, including the largest, a 2.1 cm mass in the pancreatic head. A fourth mass in the pancreatic tail was identified via intraoperative ultrasound. All lesions were successfully enucleated and excised, and glucose levels normalized off diazoxide by post-op day 2. While the primary lesion stained for insulin and somatostatin by immunofluorescence (IF), consistent with his clinical presentation, the additional tumors expressed glucagon, somatostatin, pancreatic polypeptide, and chromogranin A but were negative for insulin. Genetic testing confirmed a pathogenic heterozygous mutation in MEN1 (c.969C>A, p.Tyr323). He had no other signs of MEN-associated comorbidities on screening. Discussion/Conclusion This case demonstrates that young patients with MEN1 can present with multifocal NETs. These NETs may have polyhormonal expression patterns despite a clinical presentation consistent with one primary hormone. Our patient had clinical symptoms and laboratory evaluation consistent with an insulinoma but was found to have four NETs, each with different IF staining patterns. Advanced pre-operative and intraoperative imaging is important to identify and treat all present NETs. Moreover, serum hormone levels pre- and post-treatment could help evaluate whether NETs are actively secreting hormones into the bloodstream or simply expressing them within the pancreas. Finally, this case highlights the importance of genetic testing for MEN1 in all young patients with insulinomas.

Histogram Analysis of Apparent Diffusion Coefficient Maps Provides Genotypic and Pretreatment Phenotypic Information in Pediatric and Young Adult Rhabdomyosarcoma.

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 ×10-6mm2/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.

Performance Analysis in Children of Traditional and Deep Learning CT Lung Nodule Computer-Aided Detection Systems Trained on Adults.

BACKGROUND. Although primary lung cancer is rare in children, chest CT is commonly performed to assess for lung metastases in children with cancer. Lung nodule computer-aided detection (CAD) systems have been designed and studied primarily using adult training data, and the efficacy of such systems when applied to pediatric patients is poorly understood. OBJECTIVE. The purpose of this study was to evaluate in children the diagnostic performance of traditional and deep learning CAD systems trained with adult data for the detection of lung nodules on chest CT scans and to compare the ability of such systems to generalize to children versus to other adults. METHODS. This retrospective study included pediatric and adult chest CT test sets. The pediatric test set comprised 59 CT scans in 59 patients (30 boys, 29 girls; mean age, 13.1 years; age range, 4-17 years), which were obtained from November 30, 2018, to August 31, 2020; lung nodules were annotated by fellowship-trained pediatric radiologists as the reference standard. The adult test set was the publicly available adult Lung Nodule Analysis (LUNA) 2016 subset 0, which contained 89 deidentified scans with previously annotated nodules. The test sets were processed through the traditional FlyerScan (github.com/rhardie1/FlyerScanCT) and deep learning Medical Open Network for Artificial Intelligence (MONAI; github.com/Project-MONAI/model-zoo/releases) lung nodule CAD systems, which had been trained on separate sets of CT scans in adults. Sensitivity and false-positive (FP) frequency were calculated for nodules measuring 3-30 mm; nonoverlapping 95% CIs indicated significant differences. RESULTS. Operating at two FPs per scan, on pediatric testing data FlyerScan and MONAI showed significantly lower detection sensitivities of 68.4% (197/288; 95% CI, 65.1-73.0%) and 53.1% (153/288; 95% CI, 46.7-58.4%), respectively, than on adult LUNA 2016 subset 0 testing data (83.9% [94/112; 95% CI, 79.1-88.0%] and 95.5% [107/112; 95% CI, 90.0-98.4%], respectively). Mean nodule size was smaller (p < .001) in the pediatric testing data (5.4 ± 3.1 [SD] mm) than in the adult LUNA 2016 subset 0 testing data (11.0 ± 6.2 mm). CONCLUSION. Adult-trained traditional and deep learning-based lung nodule CAD systems had significantly lower sensitivity for detection on pediatric data than on adult data at a matching FP frequency. The performance difference may relate to the smaller size of pediatric lung nodules. CLINICAL IMPACT. The results indicate a need for pediatric-specific lung nodule CAD systems trained on data specific to pediatric patients.

Technical and Diagnostic Performance of Rapid MRI for Evaluation of Appendicitis in a Pediatric Emergency Department.

RATIONALE AND OBJECTIVES: Rationale MRI is increasingly used for diagnosis of pediatric appendicitis in the emergency care setting. Scan and room time are important to optimize workflow, especially in situations with limited MRI availability and accessibility. To describe our process to optimize and implement a rapid, non-contrast, free-breathing MRI protocol for appendicitis and to characterize the accuracy for diagnosis. MATERIALS AND METHODS: Initial MRI protocol implemented for suspected appendicitis included five respiratory-triggered axial and coronal single shot fast spin echo (SSFSE) and radial T2W sequences with and without fat suppression (FS). This protocol was reassessed based on image quality. Subsequently, the coronal and axial T2W SSFSE FS sequence were removed. A three-sequence exam comprised the rapid MRI appendicitis protocol used for the remainder of the study. All examinations were performed on a 1.5 T scanner. For all examinations, the following information was obtained: scan time, diagnosis of appendicitis (by radiology report), surgery and pathology reports, and alternative diagnoses documented in radiology reports. MRI examination scan durations were compared between the five-sequence and three-sequence protocols, with Mann-Whitney U test, and proportions of false diagnoses were compared to Fisher's exact test. RESULTS: 216 examinations were performed; 21 and 195 performed with five- and three-sequence protocol, respectively. The median duration of the five-sequence protocol was 20 (16.9-23.5) minutes vs.11 (9.5-13) minutes for the three sequence protocol (p < 0.0001). The majority (n = 157, 80.5% of 195) of examinations were performed in a goal time of< 15 min. 23 examinations took longer than 20 min (n = 12, n = 11 for the three- and five-sequence protocols, respectively) were due to repeat sequences or addition of non-standard sequences (requested by interpreting radiologist). 27 (90%) of the 30 reported positive cases were confirmed by pathology. There were three false-positive diagnoses (all three-sequence protocol exams) and one false-negative diagnosis (five-sequence protocol exam). Alternative diagnoses, including pancreatitis, omental infarct, masses, and ovarian pathology, were made in 42 (19%) cases. CONCLUSION: A three-sequence, non-contrast, free- breathing MRI examination can be performed in less than 15 min in the emergency department and has high diagnostic accuracy for acute appendicitis in children and young adults.

Current Recommended Estrogen Dosing for Pubertal Induction in Turner Syndrome Results in Normal Uterine Growth.

CONTEXT: Most individuals with Turner syndrome (TS) require estrogen for pubertal induction. Current estrogen dosing guidelines are based on expert consensus opinion. OBJECTIVE: Evaluate whether current international guidelines for estrogen dosing during pubertal induction of individuals with TS result in normal uterine growth. We hypothesized that uterine size in individuals with TS who reached adult estrogen dosing is smaller than in mature females without TS. METHODS: Cross-sectional study of patients with TS at the Cincinnati Center for Pediatric and Adult Turner Syndrome Care. Twenty-nine individuals (age 15-26 years) with primary ovarian insufficiency who reached adult estrogen dosing (100 µg of transdermal or 2 mg of oral 17ß-estradiol) were included. Comparison of uterine measurements with a published sample of 292 age-appropriate (age 15-20 years) controls without TS. Uterine length, volume, and fundal-cervical ratio (FCR) were measured. Clinical information (karyotype, Tanner staging for breast development, laboratory data) was extracted from an existing institutional patient registry. RESULTS: There was no evidence of compromise of the uterine size/configuration in the TS cohort compared with the controls; in fact, uterine length, mean 7.7 cm (±1.3) vs 7.2 cm (±1.0) (P = .03), and volume, mean 60.6 cm3 (±26.6) vs 50.5 cm3 (±20.5) (P = .02), were both larger in individuals with TS. CONCLUSION: Current international guidelines for hormone replacement using 17ß-estradiol in individuals with TS appear adequate to allow for normal uterine growth by the end of pubertal induction.

Pancreas volumes in pediatric patients following index acute pancreatitis and acute recurrent pancreatitis.

BACKGROUND/OBJECTIVES: Pancreas volume derived from imaging may objectively reveal volume loss relevant to identifying sequelae of acute pancreatitis (AP) and ultimately diagnosing chronic pancreatitis (CP). The purposes of this study were to: (1) quantify pancreas volume by imaging in children with either (a) a single episode of AP or (b) acute recurrent pancreatitis (ARP), and (2) compare these volumes to normative volumes. METHODS: This retrospective study was institutional review board approved. A single observer segmented the pancreas (3D Slicer; slicer.org) on n = 30 CT and MRI exams for 23 children selected from a prospective registry of patients with either an index attack of AP or with ARP after a known index attack date. Patients with CP were excluded. Segmented pancreas volumes were compared to published normal values. RESULTS: Mean pancreas volumes normalized to body surface area (BSA) in the index AP and ARP groups were 38.2 mL/m2 (range: 11.8-73.5 mL/m2) and 27.9 mL/m2 (range: 8.0-69.2 mL/m2) respectively. 43 % (6/14) of patients post-AP had volumes below the 25th percentile, 1 (17 %) of which was below the 5th percentile (p = 0.3027 vs. a normal distribution). Post-ARP, 44 % (7/16) of patients had volumes below the 5th percentile (p < 0.001). CONCLUSIONS: A significant fraction (40 %) of children with ARP have pancreas volumes <5th percentile for BSA even in the absence of CP. A similar, but not statistically significant, fraction have pancreas volumes <25th percentile after an index attack of AP. Pancreatic parenchymal volume deserves additional investigation as an objective marker of parenchymal damage from acute pancreatitis and of progressive pancreatitis in children.

Patient Preferences in Diagnostic Imaging: A Scoping Review.

BACKGROUND: As new diagnostic imaging technologies are adopted, decisions surrounding diagnostic imaging become increasingly complex. As such, understanding patient preferences in imaging decision making is imperative. OBJECTIVES: We aimed to review quantitative patient preference studies in imaging-related decision making, including characteristics of the literature and the quality of the evidence. METHODS: The Pubmed, Embase, EconLit, and CINAHL databases were searched to identify studies involving diagnostic imaging and quantitative patient preference measures from January 2000 to June 2022. Study characteristics that were extracted included the preference elicitation method, disease focus, and sample size. We employed the PREFS (Purpose, Respondents, Explanation, Findings, Significance) checklist as our quality assessment tool. RESULTS: A total of 54 articles were included. The following methods were used to elicit preferences: conjoint analysis/discrete choice experiment methods (n = 27), contingent valuation (n = 16), time trade-off (n = 4), best-worst scaling (n = 3), multicriteria decision analysis (n = 3), and a standard gamble approach (n = 1). Half of the studies were published after 2016 (52%, 28/54). The most common scenario (n = 39) for eliciting patient preferences was cancer screening. Computed tomography, the most frequently studied imaging modality, was included in 20 studies, and sample sizes ranged from 30 to 3469 participants (mean 552). The mean PREFS score was 3.5 (standard deviation 0.8) for the included studies. CONCLUSIONS: This review highlights that a variety of quantitative preference methods are being used, as diagnostic imaging technologies continue to evolve. While the number of preference studies in diagnostic imaging has increased with time, most examine preventative care/screening, leaving a gap in knowledge regarding imaging for disease characterization and management.

Isosorbide DiNitrate Effect on Hemodynamic Profile, Liver Stiffness, and Exercise Tolerance in Fontan Circulation (The NEET Clinical Trial).

After Fontan operation, decreased venous capacitance and venoconstriction are adaptive mechanisms to maintain venous return and cardiac output. The consequent higher venous pressure may adversely impact end-organ function, exercise capacity and result in worse clinical outcomes. This pilot study evaluated the safety and effect of isosorbide dinitrate (ISDN), a venodilator, on exercise capacity, peripheral venous pressure (PVP), and liver stiffness in patients with Fontan circulation. In this prospective single-arm trial, 15 individuals with Fontan circulation were evaluated at baseline and after 4 weeks of therapeutic treatment with ISDN. Primary aims were to assess the safety of ISDN and the effect on maximal exercise. We also aimed to evaluate the effect of ISDN on ultrasound-assessed liver stiffness, markers of submaximal exercise, and PVP at rest and peak exercise. Repeated measures t-tests were used to assess change in variables of interest in response to ISDN. Mean age was 23.5 ± 9.2 years (range 11.2-39.0 years), and 10/15 (67%) were male. There was no statistically significant change in peak VO2 (1401 ± 428 to 1428 ± 436 mL/min, p = 0.128), but VO2 at the anaerobic threshold increased (1087 ± 313 to 1115 ± 302 mL/min, p = 0.03). ISDN was also associated with a lower peak exercise PVP (22.5 ± 4.5 to 20.6 ± 3.0 mmHg, p = 0.015). Liver stiffness was lower with ISDN, though the difference was not statistically significant (2.3 ± 0.4 to 2.1 ± 0.5 m/s, p = 0.079). Of the patients completing the trial, mild headache was common (67%), but there were no major adverse events. Treatment with ISDN for 4 weeks is well-tolerated in patients with a Fontan circulation. ISDN is associated with an increase in VO2 at anaerobic threshold, lower peak PVP, and a trend toward lower liver stiffness. Larger, longer duration studies will be necessary to define the impact of ISDN on clinical outcomes in the Fontan circulation.Clinical Trial Registration: URL: https://clinicaltrials.gov . Unique identifier: NCT04297241.

Image or scope: Magnetic resonance imaging and endoscopic testing for exocrine and endocrine pancreatic insufficiency in children.

OBJECTIVES: We sought to evaluate associations between Magnetic Resonance Imaging (MRI) findings, exocrine pancreatic insufficiency (EPI) and endocrine insufficiency (prediabetes or diabetes) in children. METHODS: This was a retrospective study that included patients<21 years of age who underwent MRI and endoscopic pancreatic function testing (ePFT; reference standard for pancreatic exocrine function) within 3 months. MRI variables included pancreas parenchymal volume, secreted fluid volume in response to secretin, and T1 relaxation time. Data were analyzed for the full sample as well as the subset without acute pancreatitis (AP) at the time of imaging. RESULTS: Of 72 patients, 56% (40/72) were female with median age 11.4 years. A 5 mL decrease in pancreas parenchymal volume was associated with increased odds of exocrine pancreatic dysfunction by both ePFT (OR = 1.16, p = 0.02 full sample; OR = 1.29, p = 0.01 no-AP subset), and fecal elastase (OR = 1.16, p = 0.04 full sample; OR = 1.23, p = 0.02 no-AP subset). Pancreas parenchymal volume had an AUC 0.71 (95% CI: 0.59, 0.83) for predicting exocrine pancreatic dysfunction by ePFT and when combined with sex and presence of AP had an AUC of 0.82 (95% CI: 0.72, 0.92). Regarding endocrine function, decreased pancreas parenchymal volume was associated with increased odds of diabetes (OR = 1.16, p = 0.03), and T1 relaxation time predicted glycemic outcomes with an AUC 0.78 (95% CI: 0.55-1), 91% specificity and 73% sensitivity. CONCLUSIONS: Pancreas parenchymal volume is an MRI marker of exocrine and endocrine pancreatic dysfunction in children. A model including sex, AP, and pancreas volume best predicted exocrine status. T1 relaxation time is also an MRI marker of endocrine insufficiency.

Longitudinal changes in quantitative magnetic resonance imaging metrics in children and young adults with autoimmune liver disease.

PURPOSE: To assess longitudinal changes in quantitative MRI metrics in pediatric and young adult patients with autoimmune liver disease (AILD). METHODS: This prospective, IRB-approved study included 20 children and young adults (median age = 15 years) with primary sclerosing cholangitis (PSC)/autoimmune sclerosing cholangitis (ASC) and 19 (median age = 17 years) with autoimmune hepatitis (AIH). At a field strength of 1.5-T, T2*-corrected T1 mapping (cT1), 3D fast spin-echo MRCP, and 2D gradient recalled echo MR elastography (MRE) were performed at baseline, one year, and two years. cT1 and quantitative MRCP were processed using LiverMultiScan and MRCP + , respectively (Perspectum Ltd, Oxford, UK). Linear mixed models were used to assess longitudinal changes in quantitative MRI metrics. Spearman rank-order correlation was used to assess relationships between changes in quantitative MRI metrics. RESULTS: Changes in quantitative MRI metrics greater than established repeatability coefficients were measured in six (cT1) and five (MRE) patients with PSC/ASC as well as in six patients (cT1 and MRE) with AIH, although linear mixed models identified no significant changes for the subgroups as a whole. For PSC/ASC, there were positive correlations between change in liver stiffness and changes in bile duct strictures (ρ = 0.68; p = 0.005) and bile duct dilations (ρ = 0.70; p = 0.004) between baseline and Year 2. CONCLUSION: On average, there were no significant changes in quantitative MRI metrics over a two-year period in children and young adults with AILD. However, worsening cholangiopathy was associated with increasing liver stiffness by MRE in patients with PSC/ASC.

Impact of coronavirus disease 2019 on pediatric intestinal intussusception in the United States.

BACKGROUND: Masking and social distancing to mitigate the spread of the SARS-CoV-2 (severe acute respiratory syndrome coronavirus 2) virus curbed the spread of other viruses. Given a potential link between viral illnesses and ileocolic intussusception, the purpose of this study is to characterize trends in incidence, diagnosis and management of pediatric intussusception in the United States in the context of the coronavirus disease 2019 (COVID-19) pandemic. MATERIALS AND METHODS: This cross-sectional retrospective study used the Pediatric Hospital Information System and included children (ages 0-17 years) with a primary diagnosis of intussusception (ICD-10 [International Classification of Diseases, Tenth Revision]: K56.1) from January 2018 to December 2021. Descriptive statistics and chi-square analyses were used to characterize and compare proportions pre-COVID (2018 and 2019) to 2020 and 2021. RESULTS: Eight thousand one hundred forty-three encounters met inclusion criteria. Intussusception diagnoses declined in 2020 (n = 1,480) compared to 2019 (n = 2,321) and 2018 (n = 2,171) but returned to pre-COVID levels in 2021 (n = 2,171). Patient age was similar across years (mean age in years: 2018: 2.3; 2019: 2.1; 2020: 2.3; 2021: 2.3). There was no significant change in the proportion of patients who underwent imaging in 2020 (96% [1,415/1,480]) compared to the other years in the study (2018: 96% [2,093/2,171], P = 0.21; 2019: 97% [2,253/2,321], P = 0.80; 2021: 96% [1,415/1,480], P = 0.85). There was a statistically significant but minimal increase in the proportion of cases treated with surgery in 2020 compared to 2019 (2020: 17.8% vs. 2019: 15%, P = 0.02); however, this was not replicated in the pairwise comparison of 2020 to 2018 (2020: 17.8% vs. 2018: 16.4%, P = 0.23). There was a statistically significant increase in the proportion of cases treated with surgery in 2020 compared to 2021 (2020: 17.8% vs. 2021: 14%, P = 0.001). CONCLUSION: Pediatric intussusception diagnoses decreased at a national level in 2020 compared to previous years, with a rebound increase in 2021. This may reflect a secondary benefit of public health interventions imposed to curb the spread of COVID-19.

Imaging of pediatric pancreas tumors: A COG Diagnostic Imaging Committee/SPR Oncology Committee White Paper.

Primary pancreatic tumors in children are rare with an overall age-adjusted incidence of 0.018 new cases per 100,000 pediatric patients. The most prevalent histologic type is the solid pseudopapillary neoplasm, followed by pancreatoblastoma. This paper describes relevant imaging modalities and presents consensus-based recommendations for imaging at diagnosis and follow-up.

Computed tomography-based measurements of normative liver and spleen volumes in children.

BACKGROUND: Quantification of organ size has utility in clinical care and research for diagnostics, prognostics and surgical planning. Volumetry is regarded as the best measure of organ size and change in size over time. Scarce reference values exist for liver and spleen volumes in healthy children. OBJECTIVE: To report liver and spleen volumes for a sample of children defined by manual segmentation of contrast-enhanced CT images with the goal of defining normal values and thresholds that might indicate disease. MATERIALS AND METHODS: This retrospective study included clinically acquired contrast-enhanced CTs of the abdomen/pelvis for children and adolescents imaged between January 2018 and July 2021. Liver and spleen volumes were derived through manual segmentation of CTs reconstructed at 2.5-, 3- or 5-mm slice thickness. A subset of images (5%, n=16) was also segmented using 0.5-mm slice thickness reconstructions to define agreement based on image slice thickness. We used Pearson correlation and multivariable regression to assess associations between organ volumes and patient characteristics. We generated reference intervals for the 5th, 25th, 50th (median), 75th and 95th percentiles for organ volumes as a function of age and weight using quantile regression models. Finally, we calculated Bland-Altman plots and intraclass correlation coefficients (ICC) to quantify agreement. RESULTS: We included a total of 320 children (mean age ± standard deviation [SD] = 9±4.6 years; mean weight 38.1±18.8 kg; 160 female). Liver volume ranged from 340-2,002 mL, and spleen volume ranged from 28-480 mL. Patient weight (kg) (ß=12.5), age (months) (ß=1.7) and sex (female) (ß = -35.3) were independent predictors of liver volume, whereas patient weight (kg) (ß=2.4) and age (months) (ß=0.3) were independent predictors of spleen volume. There was excellent absolute agreement (ICC=0.99) and minimal absolute difference (4 mL) in organ volumes based on reconstructed slice thickness. CONCLUSION: We report reference liver and spleen volumes for children without liver or spleen disease. These results provide reference ranges and potential thresholds to identify liver and spleen size abnormalities that might reflect disease in children.

Radioiodine treatment of pediatric Graves disease: a multicenter review.

BACKGROUND: There is no standardized approach to iodine-131 (I-131) therapy of hyperthyroidism in pediatric Graves disease. This prevents systematic study of outcomes. OBJECTIVE: To characterize current radioiodine dosing and define therapeutic outcomes at multiple institutions that use ultrasound to measure thyroid size to guide I-131 ablation of Graves disease. MATERIALS AND METHODS: This was a retrospective cohort study conducted at three institutions. The three sites collected demographic data, thyroid volume measured by ultrasound (mL), pre-ablation radioiodine uptake, I-131 activity administered, and outcomes at 6 and 12 months for children younger than 18 years of age treated with I-131 between November 2004 and October 2019. Comparisons of continuous variables were performed using the Mann-Whitney U test. RESULTS: Sixty-nine patients (mean age: 14.5±2.5 years) were included, 59 (85.5%) of whom were female. The mean administered I-131 radioiodine activity was 12.5 mCi (463 MBq) (range: 3.8-29.9 mCi [141-1,106 MBq]). At 6 months post-ablation, 54 (80.5% of 67) patients were hypothyroid, 8 (11.9% of 67) were euthyroid and 5 were hyperthyroid. Two of the five hyperthyroid patients had become euthyroid at 12 months. At 12 months, 1 previously euthyroid patient was hyperthyroid. Administered activity per mL of thyroid tissue adjusted for 24-h uptake was lower (0.18 mCi [6.7 MBq] x %/mL vs. 0.31 mCi [11.5 MBq] x %/mL, P=0.0054) for patients who remained hyperthyroid at 6 months. CONCLUSION: There is substantial variability in administered activity for radioiodine ablation of Graves disease in children. Efforts to standardize practice should start by standardizing administered activity guided by measurement of thyroid size by ultrasound. Our results and those of previous studies suggest the need for administered activities ≥0.25 mCi [9.3 MBq] x %/mL of thyroid tissue.

Imaging of pediatric thyroid tumors: A COG Diagnostic Imaging Committee/SPR Oncology Committee White Paper.

Pediatric thyroid cancer is rare in children; however, incidence is increasing. Papillary thyroid cancer and follicular thyroid cancer are the most common subtypes, comprising about 90% and 10% of cases, respectively. This paper provides consensus imaging recommendations for evaluation of pediatric patients with thyroid cancer at diagnosis and during follow-up.