[Imaging of congenital heart defects with a focus on magnetic resonance imaging and computed tomography]. / Bildgebung angeborener Herzfehler mit Fokus auf Magnetresonanz- und Computertomographie.

CLINICAL ISSUE: Due to advances in diagnostics and therapy, the survival rate of patients with congenital heart defects is continuously increasing. The aim of this review is to compare various imaging modalities that are used in the diagnosis of congenital heart defects. METHODS: Transthoracic echocardiography is the imaging method of choice in the presence of a congenital heart defect because of its wide availability and non-invasiveness. It can be complemented by transesophageal echocardiography, cardiac catheterization, computed tomography (CT), and magnetic resonance imaging (MRI) of the heart and vessels close to the heart. METHODICAL INNOVATIONS: The radiation exposure of CT examinations of the heart is continuously decreasing because of improved technologies. MRI is also being continuously optimized, e.g., by the acquisition of MR angiographies without contrast medium application or a thin three-dimensional (3D) visualization of the entire heart with the possibility of reconstruction in all spatial planes (whole-heart technique) as well as 2D to 4D flow. PRACTICAL RECOMMENDATION: Due to the complexity of congenital heart defects and the variety of possible pathologies, the choice of imaging modality and its exact performance has to be coordinated in an interdisciplinary context and individually adapted.

Regional Pulmonary Morphology and Function: Photon-counting CT Assessment.

Background Experience with functional CT in the lungs without additional equipment in clinical routine is limited. Purpose To report initial experience and evaluate the robustness of a modified chest CT protocol and photon-counting CT (PCCT) for comprehensive analysis of pulmonary vasculature, perfusion, ventilation, and morphologic structure in a single examination. Materials and Methods In this retrospective study, consecutive patients with clinically indicated CT for various known and unknown pulmonary function impairment (six subgroups) were included between November 2021 and June 2022. After administration of an intravenous contrast agent, inspiratory PCCT was followed by expiratory PCCT after a delay of 5 minutes. Advanced automated postprocessing was performed, and CT-derived functional parameters were calculated (regional ventilation, perfusion, late contrast enhancement, and CT angiography). Mean intravascular contrast enhancement in the mediastinal vessels and radiation dose were determined. Using analysis of variance, the mean values of lung volumes, attenuation, ventilation, perfusion, and late contrast enhancement were tested for differences between subgroups of patients. Results In 166 patients (mean age, 63.2 years ± 14.2 [SD]; 106 male patients), all CT-derived parameters could be acquired (84.7% success rate; 166 of 196 patients). At the inspiratory examination, mean density was 325 HU in the pulmonary trunk, 260 HU in the left atrium, and 252 HU in the ascending aorta. The mean dose-length product for inspiration and expiration was 110.32 mGy · cm and 109.47 mGy · cm, respectively; the mean CT dose index for inspiration and expiration was 3.22 mGy and 3.09 mGy, respectively (less than the mean total radiation dose of 8-12 mGy, which is diagnostic reference level). Significant differences (P < .05) between the subgroups were found for all assessed parameters. Visual inspection allowed for voxelwise assessment of morphologic structure and function. Conclusion The proposed PCCT protocol allowed for a dose-efficient and robust simultaneous evaluation of pulmonary morphologic structure, ventilation, vasculature, and parenchymal perfusion in a procedure requiring advanced software but no additional hardware. © RSNA, 2023.

Pediatric computed tomography doses in Germany from 2016 to 2018 based on large-scale data collection.

PURPOSE: Accumulating evidence from epidemiological studies that pediatric computed tomography (CT) examinations can be associated with a small but non-zero excess risk for developing leukemia or brain tumor highlights the need to optimize doses of pediatric CT procedures. Mandatory dose reference levels (DRL) can support reduction of collective dose from CT imaging. Regular surveys of applied dose-related parameters are instrumental to decide when technological advances and optimized protocol design allow lower doses without sacrificing image quality. Our aim was to collect dosimetric data to support adapting current DRL to changing clinical practice. METHOD: Dosimetric data and technical scan parameters from common pediatric CT examinations were retrospectively collected directly from Picture Archiving and Communication Systems (PACS), Dose Management Systems (DMS), and Radiological Information Systems (RIS). RESULTS: We collected data from 17 institutions on 7746 CT series from the years 2016 to 2018 from examinations of the head, thorax, abdomen, cervical spine, temporal bone, paranasal sinuses and knee in patients below 18 years of age. Most of the age-stratified parameter distributions were lower than distributions from previously-analyzed data from before 2010. Most of the third quartiles were lower than German DRL at the time of the survey. CONCLUSIONS: Directly interfacing PACS, DMS, and RIS installations allows large-scale data collection but relies on high data-quality at the documentation stage. Data should be validated by expert knowledge or guided questionnaires. Observed clinical practice in pediatric CT imaging suggests lowering some DRL in Germany is reasonable.

[Chest X-rays in children and adolescents : Indications and limitations]. / Röntgenuntersuchungen des Thorax bei Kindern und Jugendlichen : Indikationen und Limitationen.

CLINICAL ISSUE: Chest X­ray is the most commonly performed X­ray examination in children and adolescents. The aim of this review is to present the benefit of this radiologic modality, but also its limitations. METHODS: Compared with older children, most X­ray examinations of the chest were performed in newborns. After the neonatal period, this review focusses on the diagnosis of inflammatory pulmonary changes, foreign body aspiration, detection of pulmonary nodules, and cystic fibrosis. METHODOLOGICAL INNOVATIONS: The radiation exposure of X­ray examinations is continuously decreasing due to technical innovations. However, other imaging modalities were also continuously being optimized; therefore, alternatives without radiation exposure, i.e., magnetic resonance imaging [MRI] and ultrasound, should be considered in case of specific clinical indications. PRACTICAL RECOMMENDATION: Even if the diagnostic performance of chest X­ray examinations is often minor compared to computed tomography or MRI, chest X­ray still has a high value in children and adolescents, due to its ubiquitous availability and the relatively simple acquisition.

Evaluation of neutral oral contrast agents for assessment of the small bowel at abdominal staging CT.

BACKGROUND: Although neutral oral contrast agents are widely in use, a consensus regarding a standardized protocol in abdominal staging CT does not exist. PURPOSE: To test the null hypothesis that there is no quantitative or qualitative difference between water and mannitol for evaluation of the small bowel at abdominal staging CT. MATERIAL AND METHODS: 180 patients prospectively underwent abdominal staging CT with oral administration of either 1 liter mannitol solution (n = 88) or water (n = 92). Intestinal distension was measured in 6 different segments of the small intestine. In addition, two radiologists separately evaluated diagnostic image quality with regards to luminal distension (three-point scale) in each segment and the possibility to rule out a possible underlying pathology. Quantitative and qualitative results were compared (Mann-Whitney test). RESULTS: Quantitatively, intestinal distension was comparable in all segments (p>0.05), except for the horizontal duodenum (p = 0.019). The mean luminal diameter over all intestinal segments was 19.0 mm (18.1-19.9 mm) for the water group and 18.4 mm (17.5-19.2 mm) for the mannitol group, respectively. Qualitatively, ratings were comparable for the first three segments, while distal segments were rated better using mannitol. Side effects were only observed using mannitol (n = 26; 29.5%). CONCLUSIONS: Orally administered water and mannitol solution for evaluation of the small bowel at abdominal staging CT in clinical routine resulted in comparable results for the quantitative, but not for the qualitative analysis. Looking more differentiated at the overall performance, water has advantages in terms of patient comfort, side effects and costs, and can therefore be regarded as noninferior to mannitol in this specific patient group.

KBG syndrome patient due to 16q24.3 microdeletion presenting with a paratesticular rhabdoid tumor: Coincidence or cancer predisposition?

KBG syndrome is a rare autosomal dominant disorder caused by constitutive haploinsufficiency of the ankyrin repeat domain-containing protein 11 (ANKRD11) being the result of either loss-of-function gene variants or 16q24.3 microdeletions. The syndrome is characterized by a variable clinical phenotype comprising a distinct facial gestalt and variable neurological involvement. ANKRD11 is frequently affected by loss of heterozygosity in cancer. It influences the ligand-dependent transcriptional activation of nuclear receptors and tumor suppressive function of tumor protein TP53. ANKRD11 thus serves as a candidate tumor suppressor gene and it has been speculated that its haploinsufficiency may lead to an increased cancer risk in KBG syndrome patients. While no systematic data are available, we report here on the second KBG syndrome patient who developed a malignancy. At 17 years of age, the patient was diagnosed with a left-sided paratesticular extrarenal malignant rhabdoid tumor. Genetic investigations identified a somatic truncating gene variant in SMARCB1, which was not present in the germline, and a constitutional de novo 16q24.3 microdeletion leading to a loss of the entire ANKRD11 locus. Thus, KBG syndrome was diagnosed, which was in line with the clinical phenotype of the patient. At present, no specific measures for cancer surveillance can be recommended for KBG syndrome patients. However, a systematic follow-up and inclusion of KBG syndrome patients in registries (e.g., those currently established for cancer prone syndromes) will provide empiric data to support or deny an increased cancer risk in KBG syndrome in the future.