Tips for success, managing the paediatric patient in radiology.

Paediatric radiology is a fascinating and diverse field of medicine with many opportunities to gain expertise in a range of imaging modalities and body areas. Working with children makes imaging both rewarding and challenging, due to the wide range of patient ages encountered and the inherent variation in developmental needs. This requires a patient-focussed approach to manage their anxiety and ensure cooperation of the patient and their carers. Several approaches to dealing with children have been developed including recognising individual needs, empathising with the child and carers, and involving the use of play and a range of age-relevant preparation materials. All of these make the radiology imaging environment and encounter a more effective and collaborative process. The purpose of this manuscript is to present a practical guide to overcoming these challenges, by making the child the focus of their radiological examination.

Thoracic imaging of coronavirus disease 2019 (COVID-19) in children: a series of 91 cases.

BACKGROUND: Pulmonary infection with SARS-CoV-2 virus (severe acute respiratory syndrome coronavirus 2; COVID-19) has rapidly spread worldwide to become a global pandemic. OBJECTIVE: To collect paediatric COVID-19 cases worldwide and to summarize both clinical and imaging findings in children who tested positive on polymerase chain reaction testing for SARS-CoV-2. MATERIALS AND METHODS: Data were collected by completion of a standardised case report form submitted to the office of the European Society of Paediatric Radiology from March 12 to April 8, 2020. Chest imaging findings in children younger than 18 years old who tested positive on polymerase chain reaction testing for SARS-CoV-2 were included. Representative imaging studies were evaluated by multiple senior paediatric radiologists from this group with expertise in paediatric chest imaging. RESULTS: Ninety-one children were included (49 males; median age: 6.1 years, interquartile range: 1.0 to 13.0 years, range: 9 days-17 years). Most had mild symptoms, mostly fever and cough, and one-third had coexisting medical conditions. Eleven percent of children presented with severe symptoms and required intensive unit care. Chest radiographs were available in 89% of patients and 10% of them were normal. Abnormal chest radiographs showed mainly perihilar bronchial wall thickening (58%) and/or airspace consolidation (35%). Computed tomography (CT) scans were available in 26% of cases, with the most common abnormality being ground glass opacities (88%) and/or airspace consolidation (58%). Tree in bud opacities were seen in 6 of 24 CTs (25%). Lung ultrasound and chest magnetic resonance imaging were rarely utilized. CONCLUSION: It seems unnecessary to perform chest imaging in children to diagnose COVID-19. Chest radiography can be used in symptomatic children to assess airway infection or pneumonia. CT should be reserved for when there is clinical concern to assess for possible complications, especially in children with coexisting medical conditions.

Imaging of cerebral complications of extracorporeal membrane oxygenation in infants with congenital heart disease - ultrasound with multimodality correlation.

Cranial ultrasound on neonatal intensive care units is generally performed by intensive care physicians, but radiologists often provide this crucial bedside test to children on specialist paediatric cardiac intensive care units. On a paediatric cardiac intensive care unit, complex congenital cardiac conditions are commonly encountered in both pre- and postoperative scenarios, often with the use of extracorporeal membrane oxygenation (ECMO), which both increases the risks of a number of neurologic complications and results in significant changes in vascular physiology. The aim of this pictorial essay is to discuss cranial ultrasound technique, demonstrate the changes in Doppler flow profiles resulting from veno-arterial extracorporeal membrane oxygenation and congenital cardiac conditions, and illustrate commonly encountered intracranial complications of extracorporeal membrane oxygenation support in congenital cardiac care.

Chest Radiographs for Distinguishing ADA-SCID from Other Forms of SCID.

PURPOSE: Early differentiation of adenosine deaminase deficient severe combined immunodeficiency (ADA-SCID) from other forms of SCID may initiate appropriate treatment interventions with the aim of metabolic detoxification and improved outcome. Our hypothesis was that previously described radiological features (inferior scapular angle squaring and spurring and costochondral cupping) can differentiate ADA-SCID from other forms of SCID. METHODS: Chest radiographs at clinical presentation between 2000 and 2017 of children with ADA-SCID were retrospectively included, provided that the radiological features were assessable. Random chest radiographs of children with other forms of SCID were included for comparison. Three paediatric radiologists (2 senior, 1 junior) assessed the radiographs for the specific radiological features and stated their diagnosis (ADA-SCID or non-ADA-SCID). An optimal threshold for test performance was defined using a ROC curve. RESULTS: Thirty-six patients with ADA-SCID and twenty-five patients with non-ADA-SCID were included (median age 3.8 months). The optimal threshold for test performance was at approximately < 7 months old: sensitivity 91.7%, specificity 80.7%, interreader agreement was k = 0.709, AUC 0.862. The positive likelihood ratio for scapular squaring, scapular spur, and costochondral cupping was 4.0, 54.6 and 7.8, respectively. The test was valid when performed by both senior and junior paediatric radiologists. CONCLUSION: Radiological features such as scapular spurring, scapular squaring and costochondral cupping can reliably differentiate between ADA-SCID and other forms of SCID. This is true for children aged approximately < 7 months, and this is reliable when assessed by both senior and junior paediatric radiologists.

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.

European Society of Paediatric Radiology Computed Tomography and Dose Task Force: European guidelines on diagnostic reference levels for paediatric imaging.

The recent European Council Directive 2013/59/EURATOM requires the establishment of diagnostic reference levels (DRLs) to optimise radiation dose in diagnostic and interventional radiology procedures. At the time this directive was enacted, just a few European countries had already set paediatric DRLs and many of these were outdated. For this reason, the European Commission launched a project addressing European Guidelines on Diagnostic Reference Levels for Paediatric Imaging that was awarded to a consortium led by the European Society of Radiology with the collaboration of the European Society of Paediatric Radiology and other European stakeholders involved in the radiation protection of children. The main aims of this project were to establish European DRLs to be used by countries without their own national paediatric DRLs and to provide a consistent method to establish new DRLs in the future. These European guidelines have been very recently endorsed by the European Commission and published in issue N° 185 of the Radiation Protection series. The purpose of this article is to introduce these guidelines to the wide community of paediatric radiologists.

Interstitial Lung Disease in Children Made Easier Well, Almost.

Interstitial lung disease (ILD) in pediatric patients is different from that in adults, with a vast array of pathologic conditions unique to childhood, varied modes of presentation, and a different range of radiologic appearances. Although rare, childhood ILD (chILD) is associated with significant morbidity and mortality, most notably in conditions of disordered surfactant function, with respiratory failure in 100% of neonates with surfactant protein B dysfunction and 100% mortality without lung transplantation. The authors present a summary of lung development and anatomy, followed by an organized approach, using the structure and nomenclature of the 2013 update to the chILD Research Network classification system, to aid radiologic diagnosis of chILD. Index radiologic cases with contemporaneous histopathologic findings illustrate a summary of recent imaging studies covering the full spectrum of chILD. chILD is best grouped by age at presentation from infancy (diffuse developmental disorders, lung growth abnormalities, specific conditions of unknown origin, surfactant dysfunction mutations) to later childhood (disorders of the normal host, disorders related to systemic disease processes, disorders related to immunocompromise). Appreciation of the temporal division of chILD into infant and later childhood onset, along with a sound understanding of pulmonary organogenesis and surfactant homeostasis, will aid in providing useful insight into this important group of pediatric conditions. Application of secondary lobular anatomy to interpretation of thin-section computed tomographic images is pivotal to understanding patterns of ILD and will aid in selecting and narrowing a differential diagnosis. ©RSNA, 2017.

Filamin A (FLNA) mutation-A newcomer to the childhood interstitial lung disease (ChILD) classification.

AIM: Interstitial lung disease (ILD) in infants represents a rare and heterogenous group of disorders, distinct from those occurring in adults. In recent years a new entity within this category is being recognized, namely filamin A (FLNA) mutation related lung disease. Our aims are to describe the clinical and radiological course of patients with this disease entity to aid clinicians in the prognostic counseling and management of similar patients they may encounter. METHOD: A retrospective case note review was conducted of all patients treated at our institution (a specialist tertiary referral childrens' center) for genetically confirmed FLNA mutation related lung disease. The clinical presentation, evolution, management and radiological features were recorded and a medical literature review of Medline indexed articles was conducted. RESULTS: We present a case series of four patients with interstitial lung disease and genetically confirmed abnormalities within the FLNA gene. Their imaging findings all reveal a pattern of predominantly upper lobe overinflation, coarse pulmonary lobular septal thickening and diffuse patchy atelectasis. The clinical outcomes of our patients have been variable ranging from infant death, lobar resection and need for supplemental oxygen and bronchodilators. CONCLUSION: The progressive nature of the pulmonary aspect of this disorder and need for early aggressive supportive treatment make identification crucial to patient management and prognostic counseling.

Imaging Bronchopulmonary Dysplasia-A Multimodality Update.

Bronchopulmonary dysplasia is the most common form of infantile chronic lung disease and results in significant health-care expenditure. The roles of chest radiography and computed tomography (CT) are well documented but numerous recent advances in imaging technology have paved the way for newer imaging techniques including structural pulmonary assessment via lung magnetic resonance imaging (MRI), functional assessment via ventilation, and perfusion MRI and quantitative imaging techniques using both CT and MRI. New applications for ultrasound have also been suggested. With the increasing array of complex technologies available, it is becoming increasingly important to have a deeper knowledge of the technological advances of the past 5-10 years and particularly the limitations of some newer techniques currently undergoing intense research. This review article aims to cover the most salient advances relevant to BPD imaging, particularly advances within CT technology, postprocessing and quantitative CT; structural MRI assessment, ventilation and perfusion imaging using gas contrast agents and Fourier decomposition techniques and lung ultrasound.

Evaluation of High-Pitch Ungated Pediatric Cardiovascular Computed Tomography for the Assessment of Cardiac Structures in Neonates.

PURPOSE: We evaluated a high-pitch, non-electrocardiogram-gated cardiac computed tomographic protocol, designed to image both cardiac and extracardiac structures, including coronary arteries, in a neonatal population (less than 1 year old) that was referred for congenital heart disease assessment and compared it with an optimized standard-pitch protocol in an equivalent cohort. MATERIALS AND METHODS: Twenty-nine high-pitch scans were compared with 31 age-matched, sex-matched, and weight-matched standard-pitch, dosimetrically equivalent scans. The visualization and subjective quality of both cardiac and extracardiac structures were scored by consensus between 2 trained blinded observers. Image noise, signal-to-noise and contrast-to-noise ratios, and radiation doses were also compared. RESULTS: The high-pitch protocol better demonstrated the pulmonary veins (P=0.03) and all coronary segments (all P<0.05), except the distal right coronary artery (P=0.10), with no significant difference in the visualization of the remaining cardiac or extracardiac structures. Both contrast-to-noise and signal-to-noise ratios improved due to greater vessel opacity, with significantly fewer streak (P<0.01) and motion (P<0.01) artifacts. Image noise and computed tomographic dose index were comparable across the 2 techniques; however, the high-pitch acquisition resulted in a small, but statistically significant, increase in dose-length product [13.0 mGy.cm (9.0 to 17.3) vs. 11.0 mGy.cm (9.0 to 13.0), P=0.05] due to greater z-overscanning. CONCLUSIONS: In neonates, a high-pitch protocol improves coronary artery and pulmonary vein delineation compared with the standard-pitch protocol, allowing a more comprehensive assessment of cardiovascular anatomy while obviating the need for either patient sedation or heart rate control.

How to diagnose acute appendicitis: ultrasound first.

Acute appendicitis (AA) is a common abdominal emergency with a lifetime prevalence of about 7 %. As the clinical diagnosis of AA remains a challenge to emergency physicians and surgeons, imaging modalities have gained major importance in the diagnostic work-up of patients with suspected AA in order to keep both the negative appendectomy rate and the perforation rate low. Introduced in 1986, graded-compression ultrasound (US) has well-established direct and indirect signs for diagnosing AA. In our opinion, US should be the first-line imaging modality, as graded-compression US has excellent specificity both in the paediatric and adult patient populations. As US sensitivity is limited, and non-diagnostic US examinations with non-visualization of the appendix are more a rule than an exception, diagnostic strategies and algorithms after non-diagnostic US should focus on clinical reassessment and complementary imaging with MRI/CT if indicated. Accordingly, both ionizing radiation to our patients and cost of pre-therapeutic diagnosis of AA will be low, with low negative appendectomy and perforation rates. Main Messages • Ultrasound (US) should be the first imaging modality for diagnosing acute appendicitis (AA). • Primary US for AA diagnosis will decrease ionizing radiation and cost. • Sensitivity of US to diagnose AA is lower than of CT/MRI. • Non-visualization of the appendix should lead to clinical reassessment. • Complementary MRI or CT may be performed if diagnosis remains unclear.

The radiology of diffuse interstitial pulmonary disease in children: pearls, pitfalls and new kids on the block in 2015.

Diffuse interstitial lung disease in children differs markedly from interstitial lung disease in adults and is a distinct entity. The childhood interstitial lung disease (ChILD) classification, devised in 2010 separates conditions into those occurring in infancy, and those not specific to infants, the later group containing many conditions related to systemic diseases (including connective tissue diseases and depositional/storage disorders), and conditions occurring in immunocompromised children. In this article, we briefly review normal lung growth and development. We discuss our preferred technique for imaging the lungs with computed tomography in children, and review the recent literature regarding the radiological appearance of various ChILD. We illustrate this with cases from our institution and emphasize the more recently recognised conditions including pleuroparenchymal fibroelastosis and filamin A deficiency-related lung disease.

Comparison of diagnostic performance for perinatal and paediatric post-mortem imaging: CT versus MRI.

OBJECTIVES: To compare the diagnostic yield of whole-body post-mortem computed tomography (PMCT) imaging to post-mortem magnetic resonance (PMMR) imaging in a prospective study of fetuses and children. METHODS: We compared PMCT and PMMR to conventional autopsy as the gold standard for the detection of (a) major pathological abnormalities related to the cause of death and (b) all diagnostic findings in five different body organ systems. RESULTS: Eighty two cases (53 fetuses and 29 children) underwent PMCT and PMMR prior to autopsy, at which 55 major abnormalities were identified. Significantly more PMCT than PMMR examinations were non-diagnostic (18/82 vs. 4/82; 21.9 % vs. 4.9 %, diff 17.1 % (95 % CI 6.7, 27.6; p < 0.05)). PMMR gave an accurate diagnosis in 24/55 (43.64 %; 95 % CI 31.37, 56.73 %) compared to 18/55 PMCT (32.73 %; 95 % CI 21.81, 45.90). PMCT was particularly poor in fetuses <24 weeks, with 28.6 % (8.1, 46.4 %) more non-diagnostic scans. Where both PMCT and PMMR were diagnostic, PMMR gave slightly higher diagnostic accuracy than PMCT (62.8 % vs. 59.4 %). CONCLUSION: Unenhanced PMCT has limited value in detection of major pathology primarily because of poor-quality, non-diagnostic fetal images. On this basis, PMMR should be the modality of choice for non-invasive PM imaging in fetuses and children. KEY POINTS: • Overall 17.1 % more PMCT examinations than PMMR were non-diagnostic • 28.6 % more PMCT were non-diagnostic than PMMR in fetuses <24 weeks • PMMR detected almost a third more pathological abnormalities than PMCT • PMMR gave slightly higher diagnostic accuracy when both were diagnostic.

Duplicated left pulmonary artery: an unknown disease? Three case reports and review of the literature.

We report three cases of an abnormal finding of duplicated left pulmonary artery: two of these occurring in children with Kabuki syndrome and configuring the setting of a pseudo-pulmonary sling without any clinical or cardiac cross-sectional evidence of tracheal compression. The other case instead represents duplicated left pulmonary artery with pulmonary sling caused by the retro-tracheal course of the lower left pulmonary artery associated with "Christmas Tree" arrangement of the tracheo-bronchial system. In both patients with pseudo-pulmonary sling and Kabuki syndrome, the abnormal finding was incidental during echocardiographic examination and neither of the patients required surgical repair for the condition. To the best of our knowledge, they represent the third and fourth cases in which such an anomaly of the pulmonary artery branches not forming a sling is seen in association with Kabuki syndrome. Another case represents our second experience and the second case reported in literature with duplicated left pulmonary artery in the setting of a complex tracheal anatomy. In this symptomatic patient, surgical repair of atrial septal defect and relief of the vascular ring were indicated, and the surgical repair was performed successfully at the age of 3 years.

Carpal erosions in children with juvenile idiopathic arthritis: repeatability of a newly devised MR-scoring system.

BACKGROUND: Juvenile idiopathic arthritis (JIA) is characterized by synovial inflammation, with potential risk of developing progressive joint destruction. Personalized state-of-the-art treatment depends on valid markers for disease activity to monitor response; however, no such markers exist. OBJECTIVE: To evaluate the reliability of scoring of carpal bone erosions on MR in children with JIA using two semi-quantitative scoring systems. MATERIALS AND METHODS: A total of 1,236 carpal bones (91 MR wrist examinations) were scored twice by two independent pediatric musculoskeletal radiologists. Bony erosions were scored according to estimated bone volume loss using a 0-4 scale and a 0-10 scale. An aggregate erosion score comprising the sum total carpal bone volume loss was calculated for each examination. RESULTS: The 0-4 scoring system resulted in good intra-reader agreement and moderate to good inter-observer agreement in the assessment of individual bones. Fair and moderate agreement were achieved for inter-reader and intra-reader agreement, respectively, using the 0-10 scale. Intra- and particularly inter-reader aggregate score variability were much less favorable, with wide limits of agreement. CONCLUSION: Further analysis of erosive disease patterns compared with normal subjects is required, and to facilitate the development of an alternative means of quantifying disease.

Imaging patients with renal colic-consider ultrasound first.

UNLABELLED: Renal colic is a common disease in Europe and a common cause of visit to the Emergency Department. Clinical diagnosis is usually confirmed by imaging modalities. Unenhanced computed tomography (CT) is considered the best diagnostic test due to its excellent accuracy detecting ureteral stones. However, ultrasound (US) should be considered as the primary imaging technique. It is a reproducible, non-invasive and non-expensive imaging technique, achieving accurate diagnosis in most cases without the need for radiation. Diagnosis is based on the presence of ureteral stones, but indirect findings such as the asymmetry or absence of ureteric jet, an increase of the resistive index or a colour Doppler twinkling artefact may help to suggest the diagnosis when the stone is not identified. MAIN MESSAGES: • Renal colic diagnosis is usually confirmed by imaging modalities. • Imaging diagnosis of renal colic is based on the detection of ureteral stones. • CT is the most accurate imaging technique to identify ureteral stones. • US allows correct diagnosis in most cases without using radiation. • US should be used as the first imaging modality in patients with renal colic.

Diagnostic accuracy of post mortem MRI for abdominal abnormalities in foetuses and children.

BACKGROUND: To compare the diagnostic accuracy of post-mortem magnetic resonance imaging (PMMR) specifically for abdominal pathology in foetuses and children, compared to conventional autopsy. METHODS: Institutional ethics approval and parental consent was obtained. 400 unselected foetuses and children underwent PMMR using a 1.5T Siemens Avanto MR scanner before conventional autopsy. PMMR images and autopsy findings were reported blinded to the other data respectively. RESULTS: Abdominal abnormalities were found in 70/400 (12%) autopsies. Overall sensitivity and specificity (95% confidence interval) of PMMR for abdominal pathology was 72.5% (61.0, 81.6) and 90.8% (87.0, 93.6), with positive (PPV) and negative predictive values (NPV) of 64.1% (53.0, 73.9) and 93.6% (90.2, 95.8) respectively. PMMR was good at detecting renal abnormalities (sensitivity 80%), particularly in foetuses, and relatively poor at detecting intestinal abnormalities (sensitivity 50%). Overall accuracy was 87.4% (83.6, 90.4). CONCLUSIONS: PMMR has high overall accuracy for abdominal pathology in foetuses, newborns and children. PMMR is particularly good at detecting renal abnormalities, and relatively poor at detecting intestinal abnormalities. In clinical practice, PMMR may be a useful alternative or adjunct to conventional autopsy in foetuses and children for detecting abdominal abnormalities.