Diagnostic magnetic resonance imaging characteristics of congenital mesoblastic nephroma: a retrospective multi-center International Society of Pediatric Oncology-Renal Tumor Study Group (SIOP-RTSG) radiology panel study.

BACKGROUND: Congenital mesoblastic nephroma is the most common solid renal tumor in neonates. Therefore, patients <3 months of age are advised to undergo upfront nephrectomy, whereas invasive procedures at diagnosis in patients ≥3 months of age are discouraged by the International Society of Pediatric Oncology-Renal Tumor Study Group (SIOP-RTSG). Nevertheless, discriminating congenital mesoblastic nephroma, especially from the more common Wilms tumor, solely based on imaging remains difficult. Recently, magnetic resonance imaging (MRI) has become the preferred modality. Studies focusing on MRI characteristics of congenital mesoblastic nephroma are limited. OBJECTIVE: This study aims to identify diagnostic MRI characteristics of congenital mesoblastic nephroma in the largest series of patients to date. MATERIALS AND METHODS: In this retrospective multicenter study, five SIOP-RTSG national review radiologists identified 52 diagnostic MRIs of histologically proven congenital mesoblastic nephromas. MRI was performed following SIOP-RTSG protocols, while radiologists assessed their national cases using a validated case report form. RESULTS: Patients (24/52 classic, 11/52 cellular, and 15/52 mixed type congenital mesoblastic nephroma, 2/52 unknown) had a median age of 1 month (range 1 day-3 months). Classic type congenital mesoblastic nephroma appeared homogeneous with a lack of hemorrhage, necrosis and/or cysts, showing a concentric ring sign in 14 (58.3%) patients. Cellular and mixed type congenital mesoblastic nephroma appeared more heterogeneous and were larger (311.6 and 174.2 cm3, respectively, versus 41.0 cm3 for the classic type (P<0.001)). All cases were predominantly T2-weighted isointense and T1-weighted hypointense, and mean overall apparent diffusion coefficient values ranged from 1.05-1.10×10-3 mm2/s. CONCLUSION: This retrospective international collaborative study showed classic type congenital mesoblastic nephroma predominantly presented as a homogeneous T2-weighted isointense mass with a typical concentric ring sign, whereas the cellular type appeared more heterogeneous. Future studies may use identified MRI characteristic of congenital mesoblastic nephroma for validation and for exploring the discriminative non-invasive value of MRI, especially from Wilms tumor.

MRI Characteristics of Pediatric Renal Tumors: A SIOP-RTSG Radiology Panel Delphi Study.

BACKGROUND: The SIOP-Renal Tumor Study Group (RTSG) does not advocate invasive procedures to determine histology before the start of therapy. This may induce misdiagnosis-based treatment initiation, but only for a relatively small percentage of approximately 10% of non-Wilms tumors (non-WTs). MRI could be useful for reducing misdiagnosis, but there is no global consensus on differentiating characteristics. PURPOSE: To identify MRI characteristics that may be used for discrimination of newly diagnosed pediatric renal tumors. STUDY TYPE: Consensus process using a Delphi method. POPULATION: Not applicable. FIELD STRENGTH/SEQUENCE: Abdominal MRI including T1- and T2-weighted imaging, contrast-enhanced MRI, and diffusion-weighted imaging at 1.5 or 3 T. ASSESSMENT: Twenty-three radiologists from the SIOP-RTSG radiology panel with ≥5 years of experience in MRI of pediatric renal tumors and/or who had assessed ≥50 MRI scans of pediatric renal tumors in the past 5 years identified potentially discriminatory characteristics in the first questionnaire. These characteristics were scored in the subsequent second round, consisting of 5-point Likert scales, ranking- and multiple choice questions. STATISTICAL TESTS: The cut-off value for consensus and agreement among the majority was ≥75% and ≥60%, respectively, with a median of ≥4 on the Likert scale. RESULTS: Consensus on specific characteristics mainly concerned the discrimination between WTs and non-WTs, and WTs and nephrogenic rest(s) (NR)/nephroblastomatosis. The presence of bilateral lesions (75.0%) and NR/nephroblastomatosis (65.0%) were MRI characteristics indicated as specific for the diagnosis of a WT, and 91.3% of the participants agreed that MRI is useful to distinguish NR/nephroblastomatosis from WT. Furthermore, all participants agreed that age influenced their prediction in the discrimination of pediatric renal tumors. DATA CONCLUSION: Although the discrimination of pediatric renal tumors based on MRI remains challenging, this study identified some specific characteristics for tumor subtypes, based on the shared opinion of experts. These results may guide future validation studies and innovative efforts. LEVEL OF EVIDENCE: 3 Technical Efficacy Stage: 3.

Paediatric gastrointestinal and hepatobiliary radiology: why do we need subspecialists, and what is new?

We present the case for subspecialisation in paediatric gastrointestinal and hepato-pancreatico-biliary radiology. We frame the discussion around a number of questions: What is different about the paediatric patient and paediatric gastrointestinal system? What does the radiologist need to do differently? And finally, what can be translated from these subspecialty areas into everyday practice? We cover conditions that the sub-specialist might encounter, focusing on entities such as inflammatory bowel disease and hepatic vascular anomalies. We also highlight novel imaging techniques that are a focus of research in the subspecialties, including contrast-enhanced ultrasound, MRI motility, magnetisation transfer factor, and magnetic resonance elastography.

18F-FDG PET/MRI for Staging and Interim Response Assessment in Pediatric and Adolescent Hodgkin Lymphoma: A Prospective Study with 18F-FDG PET/CT as the Reference Standard.

Treatment regimens for pediatric Hodgkin lymphoma (HL) depend on accurate staging and treatment response assessment, based on accurate disease distribution and metabolic activity depiction. With the aim of radiation dose reduction, we compared the diagnostic performance of 18F-FDG PET/MRI with a 18F-FDG PET/CT reference standard for staging and response assessment. Methods: Twenty-four patients (mean age, 15.4 y; range, 8-19.5 y) with histologically proven HL were prospectively and consecutively recruited in 2015 and 2016, undergoing both 18F-FDG PET/CT and 18F-FDG PET/MRI at initial staging (n = 24) and at response assessment (n = 21). The diagnostic accuracy of 18F-FDG PET/MRI for both nodal and extranodal disease was compared with that of 18F-FDG PET/CT, which was considered the reference standard. Discrepancies were retrospectively classified as perceptual or technical errors, and 18F-FDG PET/MRI and 18F-FDG PET/CT were corrected by removing perceptual error. Agreement with Ann Arbor staging and Deauville grading was also assessed. Results: For nodal and extranodal sites combined, corrected staging 18F-FDG PET/MRI sensitivity was 100% (95% CI, 96.7%-100%) and specificity was 99.5% (95% CI, 98.3%-99.9%). Corrected response-assessment 18F-FDG PET/MRI sensitivity was 83.3% (95% CI, 36.5%-99.1%) and specificity was 100% (95% CI, 99.2%-100%). Modified Ann Arbor staging agreement between 18F-FDG PET/CT and 18F-FDG PET/MRI was perfect (κ = 1.0, P = 0.000). Deauville grading agreement between 18F-FDG PET/MRI and 18F-FDG PET/CT was excellent (κ = 0.835, P = 0.000). Conclusion:18F-FDG PET/MRI is a promising alternative to 18F-FDG PET/CT for staging and response assessment in children with HL.

Quantitative assessment of terminal ileum motility on MR enterography in Crohn disease: a feasibility study in children.

OBJECTIVES: Investigate the relationship between quantified terminal ileal (TI) motility and histopathological activity grading, Crohn Disease MRI Index (CDMI) and faecal calprotectin. METHODS: Retrospective review of children with Crohn disease or unclassified inflammatory bowel disease, who underwent MR enterography. Dynamic imaging for 25 patients (median age 12, range 5 to 16) was analysed with a validated motility algorithm. The TI motility score was derived. The primary reference standard was TI Endoscopic biopsy Assessment of Inflammatory Activity (eAIS) within 40 days of the MR enterography. Secondary reference standards: (1) the Crohn Disease MRI Index (CDMI) and (2) faecal calprotectin levels. RESULTS: MR enterography median motility score was 0.17 a.u. (IQR 0.12 to 0.25; range 0.05 to 0.55), and median CDMI was 3 (IQR 0 to 5.5). Forty-three percent of patients had active disease (eAIS > 0) with a median eAIS score of 0 (IQR 0 to 2; range 0 to 5). The correlation between eAIS and motility was r = - 0.58 (p = 0.004, N = 23). Between CDMI and motility, r = - 0.42 (p = 0.037, N = 25). Motility score was lower in active disease (median 0.12 vs 0.21, p = 0.020) while CDMI was higher (median 5 vs 1, p = 0.04). In a subset of 12 patients with faecal calprotectin within 3 months of MR enterography, correlation with motility was r = - 0.27 (p = 0.4). CONCLUSIONS: Quantified terminal ileum motility decreases with increasing histopathological abnormality in children with Crohn disease, reproducing findings in adults. TI motility showed a negative correlation with an MRI activity score but not with faecal calprotectin levels. KEY POINTS: • It is feasible to perform MRI quantified bowel motility assessment in children using free-breathing techniques. • Bowel motility in children with Crohn disease decreases as the extent of intestinal inflammation increases. • Quantified intestinal motility may be a candidate biomarker for treatment efficacy in children with Crohn disease.

Evaluation of inter-observer variation for computed tomography identification of childhood interstitial lung disease.

Making chILD diagnoses on CT is poorly reproducible, even amongst sub-specialists. CT might best improve diagnostic confidence in a multidisciplinary team setting when augmented with clinical, functional and haematological results. http://bit.ly/327jRCw.

Correction to: European Society of Paediatric Radiology abdominal imaging task force: statement on imaging in very early onset inflammatory bowel disease.

The above article was published online with incorrect author name. The right spelling should be Damjana Kljucevsek instead of Damjana Kjucevsek. The correct name is presented here.

Acoustic shadowing in pediatric kidney stone ultrasound: a retrospective study with non-enhanced computed tomography as reference standard.

BACKGROUND: The usefulness of acoustic shadowing as a feature of pediatric kidney stone ultrasound (US) may be underestimated. OBJECTIVE: The hypothesis was that the majority of stones in children have acoustic shadowing and that its specificity is high (>90%) in pediatric kidney stones. MATERIALS AND METHODS: Our retrospective observational study included children who had undergone abdominal non-enhanced computed tomography (CT) for kidney stones in a pediatric renal stone referral centre between 2015 and 2016. US examinations prior to CT were retrospectively assessed for US features such as acoustic shadowing, twinkle artifact and stone size. These features were compared to CT as reference standard. RESULTS: Thirty-one patients (median age: 13 years, range: 1-17 years) with 77 suspected kidney stones were included. The median stone size was 5 mm (interquartile range [IQR]: 5 mm). For acoustic shadowing, sensitivity was 70% (95% confidence interval [CI] 56-80%) and specificity was 100% (95% CI 56-100%). All kidney stones with a diameter ≥9 mm demonstrated shadowing. Sensitivity for twinkle artifact was 88% (95% CI 72-96%), but specificity for twinkle artifact could not be calculated due to the lack of true negatives. All false-positive stones on US demonstrated twinkle artifact, but none showed shadowing. CONCLUSION: Acoustic shadowing was demonstrated in the majority of pediatric kidney stones. Specificity was high, but this was not significant. Twinkle artifact is a sensitive US tool for detecting (pediatric) kidney calculi, but with a risk of false-positive findings.

European Society of Paediatric Radiology abdominal imaging task force: statement on imaging in very early onset inflammatory bowel disease.

Very early onset inflammatory bowel disease (VEO-IBD) is defined as disease presenting before the age of 6. These children require a tailored imaging approach because conventional imaging studies can be difficult to perform at such a young age. Unlike inflammatory bowel disease in older children and adults, colonic disease predominates in VEO-IBD, and small-bowel disease is rare. Distinguishing Crohn disease from ulcerative colitis is challenging both clinically and on histology. Radiology offers the greatest utility for detecting small-bowel disease because it helps to distinguish the two main disease entities and guide clinical management. Small-bowel ultrasound is recommended as the first-line investigation because it requires relatively little preparation, is readily available and is generally well tolerated in young children. We present these recommendations, based on the current evidence for radiologic management in this group, and propose an imaging algorithm for investigating VEO-IBD.

Detecting inflammation in inflammatory bowel disease - how does ultrasound compare to magnetic resonance enterography using standardised scoring systems?

BACKGROUND: Magnetic resonance enterography (MRE) is the current gold standard for imaging in inflammatory bowel disease, but ultrasound (US) is a potential alternative. OBJECTIVE: To determine whether US is as good as MRE for the detecting inflamed bowel, using a combined consensus score as the reference standard. MATERIALS AND METHODS: We conducted a retrospective cohort study in children and adolescents <18 years with inflammatory bowel disease (IBD) at a tertiary and quaternary centre. We included children who underwent MRE and US within 4 weeks. We scored MRE using the London score and US using a score adapted from the METRIC (MR Enterography or Ultrasound in Crohn's Disease) trial. Four gastroenterologists assessed an independent clinical consensus score. A combined consensus score using the imaging and clinical scores was agreed upon and used as the reference standard to compare MRE with US. RESULTS: We included 53 children. At a whole-patient level, MRE scores were 2% higher than US scores. We used Lin coefficient to assess inter-observer variability. The repeatability of MRE scores was poor (Lin 0.6). Agreement for US scoring was substantial (Lin 0.95). There was a significant positive correlation between MRE and clinical consensus scores (Spearman's rho = 0.598, P=0.0053) and US and clinical consensus scores (Spearman's rho = 0.657, P=0.0016). CONCLUSION: US detects as much clinically significant bowel disease as MRE. It is possible that MRE overestimates the presence of disease when using a scoring system. This study demonstrates the feasibility of using a clinical consensus reference standard in paediatric IBD imaging studies.

Paediatric magnetic resonance enteroclysis under general anaesthesia - initial experience.

MR enterography is the accepted imaging reference standard for small bowel assessment in inflammatory bowel disease. There is an increasing cohort of children with inflammatory bowel disease presenting at an early age (<5 years) with severe disease. Younger children present a technical challenge for enterography because of the need for sedation/general anaesthesia to allow image optimisation and the need for oral contrast to allow adequate luminal assessment. Through our experiences, MR enteroclysis under general anaesthesia has proven to be a successful imaging technique for the work-up of these patients. In this paper, we present our institutional practice for performing MR enteroclysis under general anaesthesia.

Detecting inflammation in the unprepared pediatric colon - how reliable is magnetic resonance enterography?

BACKGROUND: Pediatric inflammatory bowel disease frequently affects the colon. MR enterography is used to assess the small bowel but it also depicts the colon. OBJECTIVE: To compare the accuracy of MR enterography and direct visualization at endoscopy in assessing the colon in pediatric inflammatory bowel disease. MATERIALS AND METHODS: We included children with inflammatory bowel disease who had undergone both MR enterography and endoscopy, and we restrospectively assessed the imaging and endoscopic findings. We scored the colonic appearance at MR using a total colon score. We then compared scores for the whole colon and for its individual segments with endoscopy and histology. RESULTS: We included 15 children. An elevated MR colonic segmental score predicted the presence of active inflammation on biopsy with a specificity of 90% (95% confidence interval [CI] 79.5-96.2%) and sensitivity of 60% (CI 40.6-77.3%); this compares reasonably with the predictive values for findings at colonoscopy - specificity 85% (CI 73.4-92.9%) and sensitivity 53.3% (CI 34.3%-71.6%). Accuracy did not change significantly with increasing bowel distension. CONCLUSION: MR-derived scores had comparable accuracy to those derived during visualization at colonoscopy for detecting biopsy-proven inflammation in our patient group. MR enterography might prove useful in guiding biopsy or monitoring treatment response. Collapse of a colonic segment did not impair assessment of inflammation.

Radiographic appearances of uncommon paediatric implants and devices.

As childhood survival of chronic illness improves, long-term implanted devices will be encountered more frequently in routine radiology. In our paediatric tertiary referral hospital, we have come across several types of implanted devices that were not confidently recognised or identified by front-line staff and were often only identified by discussion with the patient, family or clinical team. This pictorial review highlights the appearance of nonvascular devices on paediatric radiographs in order to help clinicians identify the most frequent complications and to improve awareness of these important devices.

Fusion and subtraction post-processing in body MRI.

Interpreting complex paediatric body MRI studies requires the integration of information from multiple sequences. Image processing software, some freely available, allows the radiologist to use simple and rapid post-processing techniques that may aid diagnosis. We demonstrate the use of fusion and subtraction post-processing techniques with examples from four areas of application: enterography, oncological imaging, musculoskeletal imaging and MR fistulography.