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.

Transplantation of Haploidentical TcRaß-Depleted Hematopoietic Cells Allows for Optimal Timing and Sustained Correction of the Metabolic Defect in Children With Infantile Osteopetrosis.

In osteopetrosis, osteoclast dysfunction can lead to deafness, blindness, bone marrow failure, and death. Hematopoietic cell transplantation (HCT) is currently the only curative treatment, but outcome remains disappointing. Although a rapid progression toward HCT is detrimental to prevent further progress of disease manifestations, 70% of cases lack an HLA-matched sibling and require alternative stem cell sources. We present two cases of osteopetrosis that successfully received an HCT with haploidentical TcRαß-depleted cells from one of the parents. These cases showed no further disease progression, had restoration of functional osteoclasts, and illustrate this approach to enable prompt HCT with ready available parental donors and rapid and sustained hematological, including osteoclast, recovery. © 2016 American Society for Bone and Mineral Research.

Effective dose from chest tomosynthesis in children.

Tomosynthesis (digital tomography) is a recently introduced low-dose alternative to CT in the evaluation of the lungs in patients with cystic fibrosis and pulmonary nodules. Previous studies have reported an adult effective dose of 0.12-0.13 mSv for chest tomosynthesis. The aim of this study was to determine the paediatric effective dose from the dose-area-product. During a 3-y period, 38 children with cystic fibrosis and 36 paediatric oncology patients were examined with chest tomosynthesis, totally 169 posteroanterior and 17 anteroposterior examinations (40 boys and 34 girls, mean age 13.7 y, range 7-20 y). Using recently reported paediatric chest tomosynthesis conversion factors (0.23-1.09 mSv Gy cm(-2)) corrected for sex, age and energy, the mean posteroanterior effective dose calculated was 0.17 mSv; using the proposed simplified conversion factors of 0.6 (8-10 y), 0.4 (11-14 y) and 0.3 mSv Gy cm(-2) (15-19 y), the mean posteroanterior effective dose calculated was 0.15 mSv. As the difference in the calculated effective dose was minor, it is recommendable to use the simplified conversion factors. Using the conversion factor for adult chest tomosynthesis (0.26 mSv Gy cm(-2)), the mean effective dose was 0.11 mSv. Anteroposterior exposures had considerably higher effective dose. By using conversion factors adapted for children, the calculated risks from radiologic procedures will be more accurate.

Radiography, tomosynthesis, CT and MRI in the evaluation of pulmonary cystic fibrosis: an untangling review of the multitude of scoring systems.

OBJECTIVE: The first radiographic scoring system for pulmonary cystic fibrosis was presented in 1958. Since then a multitude of scoring systems for radiography and computed tomography (CT) have been presented, recently also for tomosynthesis and magnetic resonance imaging (MRI). The aim of the current review was to analyse and compare the plethora of scoring systems for cystic fibrosis, especially regarding which scoring components are considered most important. METHODS: Four scoring systems for chest radiography, one for tomosynthesis, eight for CT and one for MRI were compared regarding components evaluated and their terminology; the areas scored; scoring levels; the weighting of each component in percentage of the total score; and the calculations for the final score. RESULTS: In most radiological scoring systems the lungs are evaluated for increased volume, bronchial wall thickening, bronchiectasis, mucus plugging, atelectasis and consolidation. In addition, for instance abscesses, bullae, septal thickening, mosaic perfusion, ground glass opacities and air trapping are evaluated in some CT scoring systems. Pleural affection and perfusion defects are scored on MRI. CONCLUSIONS: Bronchiectasis alone, or in combination with mucus plugging, is given the highest weighting in most scoring systems and is thus commonly considered to be the most significant finding when evaluating cystic fibrosis lung disease. TEACHING POINTS: • Scoring of examinations is used for comparison of outcome in studies. • Scoring of examinations can also be used for monitoring disease progression. • Cystic fibrosis can be scored on radiography, tomosynthesis, CT or MRI. • The typical imaging findings of cystic fibrosis depend on the imaging modality used. • Bronchiectasis is commonly considered the most significant finding when scoring cystic fibrosis.

Conversion factors for estimation of effective dose in paediatric chest tomosynthesis.

For chest tomosynthesis in adults, a conversion factor of 0.26 mSv/Gy cm² has been reported for calculating the effective dose from the registered dose-area-product. The aim of this study was to determine conversion factors for chest tomosynthesis in children. Using the Monte Carlo-based computer software PCXMC 2.0, simulations were performed on modified phantoms for males and females aged 8-19 y, in the posteroanterior and anteroposterior projection, with energies 80-140 kV and copper filtration 0.1-0.3 mm. Resulting conversion factors ranged between 0.23 and 1.09 mSv/Gy cm², decreased with patient age, were significantly higher in the anteroposterior projection and increased with increased energy or copper filtration. To avoid an underestimation of effective dose in children, it is recommended to use age-dependent conversion factors. As a simplified approach, three conversion factors might be used for posteroanterior chest tomosynthesis and radiography in children, namely 0.6 (8-10 y), 0.4 (11-14 y) and 0.3 mSv/Gy cm² (15-19 y).

Visualisation of the rectoanal inhibitory reflex with a modified contrast enema in children with suspected Hirschsprung disease.

BACKGROUND: Patients with Hirschsprung disease lack the normal rectoanal inhibitory reflex, which can be studied with anorectal manometry or US. OBJECTIVE: To see whether the rectoanal inhibitory reflex could be visualised with a modified contrast enema, thereby increasing the diagnostic accuracy of the contrast enema and reducing the number of rectal biopsies. MATERIALS AND METHODS: Fifty-nine boys and 42 girls (median age, 12 months) with suspected Hirschsprung disease were examined with a modified contrast enema, supplemented with two injections of cold, water-soluble contrast medium, to induce the reflex. Two paediatric radiologists evaluated the anonymised examinations in consensus. The contrast enema findings were correlated with the results of rectal biopsy or clinical follow-up. RESULTS: Five boys and one girl (median age, 7.5 days) were diagnosed with Hirschsprung disease. The negative predictive value of the rectoanal inhibitory reflex was 100%. A contrast enema with signs of Hirschsprung disease in combination with an absent rectoanal inhibitory reflex had the specificity of 98% and sensitivity of 100% for Hirschsprung disease. CONCLUSION: The modified contrast enema improves the radiological diagnosis of Hirschsprung disease. By demonstrating the rectoanal inhibitory reflex in children without Hirschsprung disease, we can reduce the proportion of unnecessary rectal biopsies.

Description and validation of a scoring system for tomosynthesis in pulmonary cystic fibrosis.

OBJECTIVES: To design and validate a scoring system for tomosynthesis (digital tomography) in pulmonary cystic fibrosis. METHODS: A scoring system dedicated to tomosynthesis in pulmonary cystic fibrosis was designed. Three radiologists independently scored 88 pairs of radiographs and tomosynthesis examinations of the chest in 60 patients with cystic fibrosis and 7 oncology patients. Radiographs were scored according to the Brasfield scoring system and tomosynthesis examinations were scored using the new scoring system. RESULTS: Observer agreements for the tomosynthesis score were almost perfect for the total score with square-weighted kappa >0.90, and generally substantial to almost perfect for subscores. Correlation between the tomosynthesis score and the Brasfield score was good for the three observers (Kendall's rank correlation tau 0.68, 0.77 and 0.78). Tomosynthesis was generally scored higher as a percentage of the maximum score. Observer agreements for the total score for Brasfield score were almost perfect (square-weighted kappa 0.80, 0.81 and 0.85). CONCLUSIONS: The tomosynthesis scoring system seems robust and correlates well with the Brasfield score. Compared with radiography, tomosynthesis is more sensitive to cystic fibrosis changes, especially bronchiectasis and mucus plugging, and the new tomosynthesis scoring system offers the possibility of more detailed and accurate scoring of disease severity. KEY POINTS: Tomosynthesis is more sensitive than conventional radiography for pulmonary cystic fibrosis changes. The radiation dose from chest tomosynthesis is low compared with computed tomography. Tomosynthesis may become useful in the regular follow-up of patients with cystic fibrosis.

Tomosynthesis in pulmonary cystic fibrosis with comparison to radiography and computed tomography: a pictorial review.

The purpose of this pictorial review is to illustrate chest imaging findings of cystic fibrosis (CF) using tomosynthesis (digital tomography), in comparison to radiography and computed tomography (CT). CF is a chronic systemic disease where imaging has long been used for monitoring chest status. CT exposes the patient to a substantially higher radiation dose than radiography, rendering it unsuitable for the often needed repeated examinations of these patients. Tomosynthesis has recently appeared as an interesting low dose alternative to CT, with an effective dose of approximately 0.08 mSv for children and 0.12 mSv for adults. Tomosynthesis is performed on the same X-ray system as radiography, adding only about 1 min to the normal examination time. Typical pulmonary changes in CF such as mucus plugging, bronchial wall thickening, and bronchiectases are shown in significantly better detail with tomosynthesis than with traditional radiography. In addition, the cost for a tomosynthesis examination is low compared to CT. To reduce the radiation burden of patients with CF it is important to consider low dose alternatives to CT, especially in the paediatric population. Tomosynthesis has a lower radiation dose than CT and gives a superior visualisation of pulmonary CF changes compared to radiography. It is important to further determine the role of tomosynthesis for monitoring disease progression in CF.