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Background and objective: The most common respiratory complication of extreme prematurity is bronchopulmonary dysplasia(BPD), leading to structural lung changes and impaired respiratory outcomes. However, also extremely preterm children without BPD may show similar adverse respiratory outcomes. There is need for a safe imaging modality for preterm children with and without BPD for disease severity and risk stratification assessment. Our objective was to develop a chest Magnetic Resonance Imaging(MRI) protocol in preterm children with and without BPD at school age. Methods: Nine healthy volunteers (median age 11.6(range 8.8-12.8) years), eleven preterm children with BPD (11.0(7.2-15.6) years) and nine without BPD (11.1(10.7-12.6) years) underwent MRI. MR images were scored on hypo- and hyperintense abnormalities, bronchopathy and architectural distortion. MRI data were correlated to spirometry. Ventilation and perfusion defects were analysed using Fourier Decomposition (FD) MRI. Results: On MRI, children with BPD had a higher %diseased lung (9.1 (IQR 5.9-11.6)%) compared to preterm children without BPD (3.4 (IQR 2.5-5.4)%, p1 (r=-0.40, p=0.04), FEV1/FVC (r=-0.49, p=0.009) and FEF75 (r=-0.63, pConclusion: MRI can identify structural and functional lung damage at school age in preterm children with and without BPD, showing good correlation with spirometry. We propose chest MRI as a sensitive and safe imaging method (without ionising radiation, contrast agents or the use of anaesthesia) for the long-term follow up of preterm children.

Introduction To date, no consensus has been reached on the optimal management of congenital lung abnormalities, and factors predicting postnatal outcome have not been identified. We developed an objective quantitative computed tomography (CT) scoring method, and assessed its value for clinical decision‐making. Methods Volumetric CT‐scans of all patients born with a congenital lung abnormality between January 1999 and 2018 were assessed. Lung disease was quantified using the newly‐developed congenital lung abnormality quantification (CLAQ) scoring method. In 20 equidistant axial slices, cells of a square grid were scored according to the abnormality within. The scored CT parameters were used to predict development of symptoms, and SD scores for spirometry and exercise tolerance (Bruce treadmill test) at 8 years of age. Results CT‐scans of 124 patients with a median age of 5 months were scored. Clinical diagnoses included congenital pulmonary airway malformation (49%), bronchopulmonary sequestration (27%), congenital lobar overinflation (22%), and bronchogenic cyst (1%). Forty‐four patients (35%) developed symptoms requiring surgery of whom 28 (22%) patients became symptomatic before a CT‐scan was scheduled. Lesional hyperdensity was found as an important predictor of symptom development and decreased exercise tolerance. Using receiver operating characteristic analysis, an optimal cut‐off value for developing symptoms was found at 18% total disease. Conclusion CT‐quantification of congenital lung abnormalities using the CLAQ method is an objective and reproducible system to describe congenital lung abnormalities on chest CT. The risk for developing symptoms may increase when more than a single lung lobe is affected.

Background: Magnetic Resonance Imaging (MRI) techniques to image the larynx have evolved rapidly into a promising and safe imaging modality, without need for sedation or ionizing radiation. MRI is therefore of great interest to image pediatric laryngeal diseases. Our aim was to review MRI developments relevant for the pediatric larynx and to discuss future imaging options. Methods: A systematic search was conducted to identify all morphological and diagnostic studies in which MRI was used to image the pediatric larynx, laryngeal disease, or vocal cords. Results: Fourteen articles were included: three studies on anatomical imaging of the larynx, two studies on Diffusion Weighted Imaging, four studies on vocal cord imaging and five studies on the effect of anaesthesiology on the pediatric larynx. MRI has been used for pediatric laryngeal imaging since 1991. MRI provides excellent soft tissue contrast and good visualization of vascular diseases such as haemangiomas. However, visualization of cartilaginous structures, with varying ossification during childhood, and tissue differentiation remain challenging. The latter has been partly overcome with diffusion weighted imaging (DWI), differentiating between benign and malignant masses with excellent sensitivity (94-94.4%) and specificity (91.2-100%). Vocal cord imaging evolved from static images focused on vocal tract growth to dynamic images able to detect abnormal vocal cord movement. Conclusion: MRI is promising to evaluate the pediatric larynx, but studies using MRI as diagnostic imaging modality are scarce. New static and dynamic MR imaging techniques could be implemented in the pediatric population. Further research on imaging of pediatric laryngeal diseases should be conducted.

Purpose: Computed tomography (CT) and magnetic resonance imaging (MRI) scans are used to assess and monitor several pediatric lung diseases. It is well recognized that lung volume at the moment of acquisition has a major impact on the appearance of lung parenchyma and airways. Importantly, the sensitivity of chest CT and MRI to detect bronchiectasis and gas trapping is highly dependent on adequate volume control during the image acquisition. This paper describes a feasible method to obtain accurate control of lung volume during chest imaging in pediatric patients with lung disease. Procedure: A procedure to obtain maximal respiratory manoeuvres with spirometry guidance during image acquisition for CT and MRI is described. This procedure requires training of the subject, an MRI compatible spirometer and close collaboration between a lung function scientist and the radiographer. A good to excellent target volume level for the inspiratory or expiratory scan can be achieved in around 90% of children. An important condition for this success rate is the training of the subject, executed prior to each chest CT or MRI, and instructions by the lung function scientist during the chest CT. Conclusion: Implementing lung volume guidance with a spirometer is an important and feasible step to standardize chest imaging and to optimize the diagnostic yield of chest CT and MRI in children with lung disease. Training and the collaborative effort by a lung function scientist and radiographer is the key factor for success of this procedure. Pediatr Pulmonol. 2017;52:48–56.

SummaryBackground Bronchopulmonary dysplasia (BPD) is a common respiratory complication of preterm birth and associated with long-term respiratory sequelae. Chest computed tomography (CT) is a sensitive tool to obtain insight in structural lung abnormalities and may be a predictor for later symptoms. Objectives To give an overview of chest CT scoring methods that are used to evaluate chest CT scans of BPD patients. To review which structural lung abnormalities are described in children and adults with BPD and whether these are related to clinical outcomes. Methods An extensive literature search was conducted for relevant studies on chest CT imaging in patients born preterm with BPD. Results We retrieved 316 original papers of which 16 articles and three abstracts fulfilled our inclusion criteria. Overall, we identified nine different semi-quantitative scoring methods. Chest CT scans revealed structural abnormalities in >85% of BPD patients. These abnormalities are decreased pulmonary attenuation, opacities, bronchial wall thickening, and consolidations. Some have been found to be negatively correlated with lung function and respiratory symptoms. Conclusions None of the currently described scoring systems are appropriately validated or superior over another. Future studies are needed to generate a validated and universal chest CT quantitative scoring method for patients with BPD. Pediatr Pulmonol. 2016; 51:975–986. © 2016 Wiley Periodicals, Inc.

Summary Background Computed Tomography (CT) is the gold standard to assess bronchiectasis and trapped air in cystic fibrosis (CF) lung disease, but has the disadvantage of radiation exposure. Magnetic Resonance Imaging (MRI) is a radiation free alternative. Objective To validate MRI as outcome measure by: correlating MRI scores for bronchiectasis and trapped air with clinical parameters, and by comparing those MRI scores with CT scores. Methods In patients with CF (aged 5.6–17.4 years), MRI and CT were alternated annually during routine annual check-ups between July 2007 and January 2010. Twenty-three children had an MRI performed 1 year prior to CT, 34 children had a CT 1 year prior to MRI. Bronchiectasis and trapped air were scored using the CF-MRI and CF-CT scoring system. CF-MRI scores were correlated with clinical parameters: FEV1, Pseudomonas aeruginosa, pulmonary exacerbations and patient-reported respiratory symptoms measured on the Cystic Fibrosis Questionnaire-Revised (CFQ-R), using Spearman's correlation coefficient. MRI and CT scores were compared using intra-class correlation coefficients (ICC) and Bland–Altman plots. Results Fifty-seven patients who had an MRI, CT and CFQ-R during the study period were included. CF-MRI bronchiectasis correlated with FEV1, Pseudomonas aeruginosa, pulmonary exacerbations and patient-reported respiratory symptoms. CF-MRI trapped air only correlated with FEV1 and Pseudomonas aeruginosa. ICCs between MRI and CT bronchiectasis and trapped air were 0.41 and 0.35 respectively. MRI tended to overestimate bronchiectasis compared to CT. Conclusion The associations between CF-MRI scores and several important clinical parameters further contributes to the validation of MRI. MRI provides different information than CT. Pediatr Pulmonol. © 2015 Wiley Periodicals, Inc.

For cystic fibrosis (CF) patients older than 6 years there are convincing data that suggest respiratory tract exacerbations (RTE) play an important role in the progressive loss of functional lung tissue. There is a poor understanding of the pathobiology of RTE and whether specific treatment of RTE reduces lung damage in the long term. In addition, there are limited tools available to measure the various components of CF lung disease and responses to therapy. Therefore, in order to better understand the impact of RTE on CF lung disease we need to develop sensitive measures to characterize RTE and responses to treatment; and improve our understanding of structure-function changes during treatment of RTE. In this paper we review our current knowledge of the impact of RTE on the progression of lung disease and identify strategies to improve our understanding of the pathobiology of RTE. By improving our knowledge regarding RTE in CF we will be better positioned to develop approaches to treatment that are individualized and that can prevent permanent structural damage. We suggest the development of a ventilation, perfusion, inflammation and structure (VIPS)-MRI suite that supplies the clinician with data on ventilation, inflammation, perfusion, and structure in one MRI session. VIPS-MRI could be an important step to better understand the factors that contribute to and limit treatment efficacy of RTE.

Bronchopulmonary dysplasia (BPD) is a common respiratory complication of preterm birth and associated with long-term respiratory sequelae. Chest computed tomography (CT) is a sensitive tool to obtain insight in structural lung abnormalities and may be a predictor for later symptoms.To give an overview of chest CT scoring methods that are used to evaluate chest CT scans of BPD patients. To review which structural lung abnormalities are described in children and adults with BPD and whether these are related to clinical outcomes.An extensive literature search was conducted for relevant studies on chest CT imaging in patients born preterm with BPD.We retrieved 316 original papers of which 16 articles and three abstracts fulfilled our inclusion criteria. Overall, we identified nine different semi-quantitative scoring methods. Chest CT scans revealed structural abnormalities in85% of BPD patients. These abnormalities are decreased pulmonary attenuation, opacities, bronchial wall thickening, and consolidations. Some have been found to be negatively correlated with lung function and respiratory symptoms.None of the currently described scoring systems are appropriately validated or superior over another. Future studies are needed to generate a validated and universal chest CT quantitative scoring method for patients with BPD. Pediatr Pulmonol. 2016; 51:975-986. © 2016 Wiley Periodicals, Inc.

Computed tomography (CT) and magnetic resonance imaging (MRI) scans are used to assess and monitor several pediatric lung diseases. It is well recognized that lung volume at the moment of acquisition has a major impact on the appearance of lung parenchyma and airways. Importantly, the sensitivity of chest CT and MRI to detect bronchiectasis and gas trapping is highly dependent on adequate volume control during the image acquisition. This paper describes a feasible method to obtain accurate control of lung volume during chest imaging in pediatric patients with lung disease.A procedure to obtain maximal respiratory manoeuvres with spirometry guidance during image acquisition for CT and MRI is described. This procedure requires training of the subject, an MRI compatible spirometer and close collaboration between a lung function scientist and the radiographer. A good to excellent target volume level for the inspiratory or expiratory scan can be achieved in around 90% of children. An important condition for this success rate is the training of the subject, executed prior to each chest CT or MRI, and instructions by the lung function scientist during the chest CT.Implementing lung volume guidance with a spirometer is an important and feasible step to standardize chest imaging and to optimize the diagnostic yield of chest CT and MRI in children with lung disease. Training and the collaborative effort by a lung function scientist and radiographer is the key factor for success of this procedure. Pediatr Pulmonol. 2017;52:48-56. © 2016 Wiley Periodicals, Inc.

For cystic fibrosis (CF) patients older than 6 years there are convincing data that suggest respiratory tract exacerbations (RTE) play an important role in the progressive loss of functional lung tissue. There is a poor understanding of the pathobiology of RTE and whether specific treatment of RTE reduces lung damage in the long term. In addition, there are limited tools available to measure the various components of CF lung disease and responses to therapy. Therefore, in order to better understand the impact of RTE on CF lung disease we need to develop sensitive measures to characterize RTE and responses to treatment; and improve our understanding of structure-function changes during treatment of RTE. In this paper we review our current knowledge of the impact of RTE on the progression of lung disease and identify strategies to improve our understanding of the pathobiology of RTE. By improving our knowledge regarding RTE in CF we will be better positioned to develop approaches to treatment that are individualized and that can prevent permanent structural damage. We suggest the development of a ventilation, perfusion, inflammation and structure (VIPS)-MRI suite that supplies the clinician with data on ventilation, inflammation, perfusion, and structure in one MRI session. VIPS-MRI could be an important step to better understand the factors that contribute to and limit treatment efficacy of RTE.

Computed Tomography (CT) is the gold standard to assess bronchiectasis and trapped air in cystic fibrosis (CF) lung disease, but has the disadvantage of radiation exposure. Magnetic Resonance Imaging (MRI) is a radiation free alternative.To validate MRI as outcome measure by: correlating MRI scores for bronchiectasis and trapped air with clinical parameters, and by comparing those MRI scores with CT scores.In patients with CF (aged 5.6-17.4 years), MRI and CT were alternated annually during routine annual check-ups between July 2007 and January 2010. Twenty-three children had an MRI performed 1 year prior to CT, 34 children had a CT 1 year prior to MRI. Bronchiectasis and trapped air were scored using the CF-MRI and CF-CT scoring system. CF-MRI scores were correlated with clinical parameters: FEV1 , Pseudomonas aeruginosa, pulmonary exacerbations and patient-reported respiratory symptoms measured on the Cystic Fibrosis Questionnaire-Revised (CFQ-R), using Spearman's correlation coefficient. MRI and CT scores were compared using intra-class correlation coefficients (ICC) and Bland-Altman plots.Fifty-seven patients who had an MRI, CT and CFQ-R during the study period were included. CF-MRI bronchiectasis correlated with FEV1 , Pseudomonas aeruginosa, pulmonary exacerbations and patient-reported respiratory symptoms. CF-MRI trapped air only correlated with FEV1 and Pseudomonas aeruginosa. ICCs between MRI and CT bronchiectasis and trapped air were 0.41 and 0.35 respectively. MRI tended to overestimate bronchiectasis compared to CT.The associations between CF-MRI scores and several important clinical parameters further contributes to the validation of MRI. MRI provides different information than CT.