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This article, titled "Timing of magnetic resonance imaging in pregnancy for congenital diaphragmatic hernia," examines the impact of the timing of MRI on the prediction of survival and morbidity in patients with CDH. The study, conducted in Germany and Italy, included 354 patients with isolated CDH. The results showed that the timing of MRI did not affect the prediction of survival rate or morbidity, as the observed to expected total fetal lung volume (o/e TFLV) did not change during pregnancy. Clinicians can choose any gestational age starting in the mid-second trimester for the assessment of severity and counseling. [Extracted from the article]

Purpose: To evaluate the impact of the timing of MRI on the prediction of survival and morbidity in patients with CDH, and whether serial measurements have a beneficial value., Methods: This retrospective cohort study was conducted in two perinatal centers, in Germany and Italy. It included 354 patients with isolated CDH having at least one fetal MRI. The severity was assessed with the observed-to-expected total fetal lung volume (o/e TFLV) measured by two experienced double-blinded operators. The cohort was divided into three groups according to the gestational age (GA) at which the MRI was performed (< 27, 27-32, and  > 32 weeks' gestation [WG]). The accuracy for the prediction of survival at discharge and morbidity was analyzed with receiver operating characteristic (ROC) curves. Multiple logistic regression analyses and propensity score matching examined the population for balance. The effect of repeated MRI was evaluated in ninety-seven cases., Results: There were no significant differences in the prediction of survival when the o/e TFLV was measured before 27, between 27 and 32, and after 32 WG (area under the curve [AUC]: 0.77, 0.79, and 0.77, respectively). After adjustment for confounding factors, it was seen, that GA at MRI was not associated with survival at discharge, but the risk of mortality was higher with an intrathoracic liver position (adjusted odds ratio [aOR]: 0.30, 95% confidence interval [95%CI] 0.12-0.78), lower GA at birth (aOR 1.48, 95%CI 1.24-1.78) and lower o/e TFLV (aOR 1.13, 95%CI 1.06-1.20). ROC curves showed comparable prediction accuracy for the different timepoints in pregnancy for pulmonary hypertension, the need of extracorporeal membrane oxygenation, and feeding aids. Serial measurements revealed no difference in change rate of the o/e TFLV according to survival., Conclusion: The timing of MRI does not affect the prediction of survival rate or morbidity as the o/e TFLV does not change during pregnancy. Clinicians could choose any gestational age starting mid second trimester for the assessment of severity and counseling., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

The introduction of CFTR modulator drugs like elexacaftor-tezacaftor-ivacaftor (ETI) has transformed the management of cystic fibrosis (CF), significantly improving symptoms, lung function, and quality of life, while reducing reliance on intravenous antibiotics. However, respiratory exacerbations in the CFTR modulators era remain poorly understood from both pathophysiological and clinical perspectives. We present the case of a 20-year-old Caucasian woman with CF (F508del/L1077P) who, after three years of ETI treatment, experienced a severe episode of hemoptysis, despite being almost asymptomatic in the weeks leading up to admission, requiring bronchial artery embolization. Following ETI treatment, auscultatory findings and FEV 1 changes may be less significant, making the detection of respiratory exacerbation more challenging. This highlights the need for heightened vigilance in managing such cases and underscores the challenge of diagnosing and managing exacerbations in the era of modulators. Long term real-world studies are essential to comprehend the evolving course of the disease during ETI treatment., Competing Interests: Declarations of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Prenatal assessment of lung size and liver position is essential to stratify congenital diaphragmatic hernia (CDH) fetuses in risk categories, guiding counseling, and patient management. Manual segmentation on fetal MRI provides a quantitative estimation of total lung volume and liver herniation. However, it is time-consuming and operator-dependent. In this study, we utilized a publicly available deep learning (DL) segmentation system (nnU-Net) to automatically contour CDH-affected fetal lungs and liver on MRI sections. Concordance between automatic and manual segmentation was assessed by calculating the Jaccard coefficient. Pyradiomics standard features were then extracted from both manually and automatically segmented regions. The reproducibility of features between the two groups was evaluated through the Wilcoxon rank-sum test and intraclass correlation coefficients (ICCs). We finally tested the reliability of the automatic-segmentation approach by building a ML classifier system for the prediction of liver herniation based on support vector machines (SVM) and trained on shape features computed both in the manual and nnU-Net-segmented organs. We compared the area under the classifier receiver operating characteristic curve (AUC) in the two cases. Pyradiomics features calculated in the manual ROIs were partly reproducible by the same features calculated in nnU-Net segmented ROIs and, when used in the ML procedure, to predict liver herniation (both AUC around 0.85).          Conclusion: Our results suggest that automatic MRI segmentation is feasible, with good reproducibility of pyradiomics features, and that a ML system for liver herniation prediction offers good reliability.          Trial registration: https://clinicaltrials.gov/ct2/show/NCT04609163?term=NCT04609163&draw=2&rank=1 ; Clinical Trial Identification no. NCT04609163. What is Known: • Magnetic resonance imaging (MRI) is crucial for prenatal congenital diaphragmatic hernia (CDH) assessment. It enables the quantification of the total lung volume and the extent of liver herniation, which are essential for stratifying the severity of CDH, guiding counseling, and patient management. • The manual segmentation of MRI scans is a time-consuming process that is heavily reliant upon the skill set of the operator. What is New: • MRI lung and liver automatic segmentation using the deep learning nnU-Net system is feasible, with good Jaccard coefficient values and satisfactory reproducibility of pyradiomics features compared to manual results. • A feasible ML system for predicting liver herniation could improve prenatal assessments and CDH patient management., (© 2024. The Author(s).)

Lobar atelectasis may be a complication of pulmonary exacerbations in cystic fibrosis (CF). There are no established guidelines on the management of this condition in patients with CF. Therapeutic bronchoscopy with recombinant human deoxyribonuclease (rhDNase) instillation has been described to be successful in patients not responding to conservative measures. We describe a case of a young man with CF, with previously mild impaired lung function, presenting with cough, desaturation, and worsening dyspnea, persisting for over 6 weeks, despite conservative therapy. Thoracic imaging showed right lower lobe atelectasis, which was successfully treated with bronchoscopy and instillation of rhDNase. Long-term resolution of the atelectasis was confirmed with chest magnetic resonance imaging follow-up., (© 2022 The Authors. Pediatric Pulmonology published by Wiley Periodicals LLC.)

Objective: Percutaneous transluminal renal angioplasty (PTRA), the recommended treatment in children with renovascular hypertension (RVH), often has unsatisfactory outcomes. Cutting balloons may improve the results of angioplasty in different vascular beds with complex and resistant lesions. We retrospectively analysed the effects of percutaneous cutting balloon angioplasty (PCBA) on blood pressure, cardiac mass and renal artery acceleration time in children/adolescents referred to our centre for RVH., Patients and Methods: Thirteen patients (aged 9-19 years) with renal artery stenosis (RAS) and severe hypertension were identified. RASs were focal fibromuscular (FMD) or FMD-like dysplasia (in six cases bilateral, in five associated with mid aortic syndrome). Ten patients had uncontrolled hypertension, in nine cases associated with left ventricular hypertrophy (LVH). Acceleration time was abnormal in all stenotic arteries. Eighteen PCBA were performed, in three arteries associated with stent implantation., Results: PCBA was technically successful in all individuals without major complications. In one patient, an intra-stent restenosis occurred, successfully redilated with conventional angioplasty without recurrence at 4 years distance. One year after PCBA, mean SBP and DBPs were markedly reduced from 146 ± 25 to 121 ± 10 mmHg and from 87 ± 11 to 65 ± 12 mmHg, respectively ( P  < 0.001 for both). At that time, hypertension was cured in seven children and controlled in five individuals. This favourable outcome was confirmed with ambulatory blood pressure measurement in four patients. At the latest follow-up, left ventricular mass and acceleration time were normal in all patients., Conclusion: PCBA proved to be a well tolerated and effective procedure that can be considered as an alternative to PTRA to treat hypertensive children/adolescents with recurrent or resistant RAS., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

In recent years, magnetic resonance imaging (MRI) has largely increased our knowledge and predictive accuracy of congenital diaphragmatic hernia (CDH) in the fetus. Thanks to its technical advantages, better anatomical definition, and superiority in fetal lung volume estimation, fetal MRI has been demonstrated to be superior to 2D and 3D ultrasound alone in CDH diagnosis and outcome prediction. This is of crucial importance for prenatal counseling, risk stratification, and decision-making approach. Furthermore, several quantitative and qualitative parameters can be evaluated simultaneously, which have been associated with survival, postnatal course severity, and long-term morbidity., Conclusion: Fetal MRI will further strengthen its role in the near future, but it is necessary to reach a consensus on indications, methodology, and data interpretation. In addition, it is required data integration from different imaging modalities and clinical courses, especially for predicting postnatal pulmonary hypertension. This would lead to a comprehensive prognostic assessment., What Is Known: • MRI plays a key role in evaluating the fetal lung in patients with CDH. • Prognostic assessment of CDH is challenging, and advanced imaging is crucial for a complete prenatal assessment and counseling., What Is New: • Fetal MRI has strengthened its role over ultrasound due to its technical advantages, better anatomical definition, superior fetal lung volume estimation, and outcome prediction. • Imaging and clinical data integration is the most desirable strategy and may provide new MRI applications and future research opportunities., (© 2022. The Author(s).)

Objectives: To reach a molecular diagnosis for a family with two consecutive fetuses presenting with multiple congenital anomalies., Methods: The two fetuses underwent prenatal ultrasound, autopsy, radiologic, and genetic investigation. Genetic analysis included karyotype and array-CGH for both fetuses and trio-based whole exome sequencing (WES) only for the second fetus., Results: WES results, initially focusing on recessive or dominant de novo variants, were negative.However, as a result of new relevant information regarding family history, the variant c.648&#95;651dup in the PTCH1 gene was identified as causative of the fetal phenotype., Conclusions: This case further highlights how WES data analysis and interpretation strongly rely on family history and robust genotype-phenotype correlation. This is even more relevant in the prenatal setting, where access to fetal phenotype is limited and prenatal recognition of many morbid genes is not fully explored. We also provide a detailed description of the prenatal manifestations of Basal Cell Nevus Syndrome., (© 2022 John Wiley & Sons Ltd.)

In newborns with congenital diaphragmatic hernia (CDH), the mediastinal shift caused by the herniated organs negatively affects lung development. Assessment of the fetal magnetic resonance imaging (MRI) mediastinal shift angle (MSA) was shown to have an inverse correlation with the total fetal lung volume (TFLV), being associated with neonatal survival. However, a possible association with postnatal morbidity has never been investigated. We hypothesize that the degree of the mediastinal shift could be associated with higher respiratory and cardiocirculatory impairment, requiring intensive treatments and extended hospitalization in survivors. We retrospectively consider a cohort of isolated, left-sided CDH, for whom we calculated the MSA and the observed/expected (O/E) TFLV at fetal MRI. We performed a data collection regarding inotropic or vasoactive support, treatment with pulmonary vasodilators, mechanical ventilation, and length of stay. General linear models were performed. The MSA and O/E TFLV were inversely correlated (Pearson's coefficient - 0.65, p < 0.001), and deceased patients showed higher MSA values then survivors (p = 0.011). Among survivors, an increase in MSA was associated with longer pharmacological treatments (dobutamine: p = 0.016; dopamine: p = 0.049; hydrocortisone: p = 0.003; nitric oxide: p = 0.002; sildenafil: p = 0.039; milrinone: p = 0.039; oxygen: p = 0.066), and mechanical ventilation (p = 0.005), with an increasing trend in the length of hospitalization (p = 0.089).Conclusion: The MSA indirectly reflects lung hypoplasia and is associated with a higher neonatal intensity of cares. However, further studies are needed to consolidate the results.Trial registration: The study is an exploratory post hoc analysis of the registered NeoAPACHE protocol at ClinicalTrials.gov with the identifier NCT04396028. What is Known: • In congenital diaphragmatic hernia, the lung size, liver position, and defect side are the most common prenatal prognostic parameters used in clinical practice for morbidity and mortality prediction. • Lung hypoplasia, strictly associated with lung size, is estimated by observed/expected lung to head ratio and observed/expected total fetal lung volume with prenatal ultrasound and fetal magnetic resonance imaging, respectively. What is New: • A new, faster, more straightforward, and less operator-dependent tool to assess CDH severity could be the mediastinal shift angle calculation with fetal magnetic resonance imaging. • Postnatal clinical severity, considered as a postnatal cardiovascular and respiratory impairment that indirectly reflects lung hypoplasia, is associated with an increased mediastinal shift angle calculation., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Introduction: Outcome predictions of patients with congenital diaphragmatic hernia (CDH) still have some limitations in the prenatal estimate of postnatal pulmonary hypertension (PH). We propose applying Machine Learning (ML), and Deep Learning (DL) approaches to fetuses and newborns with CDH to develop forecasting models in prenatal epoch, based on the integrated analysis of clinical data, to provide neonatal PH as the first outcome and, possibly: favorable response to fetal endoscopic tracheal occlusion (FETO), need for Extracorporeal Membrane Oxygenation (ECMO), survival to ECMO, and death. Moreover, we plan to produce a (semi)automatic fetus lung segmentation system in Magnetic Resonance Imaging (MRI), which will be useful during project implementation but will also be an important tool itself to standardize lung volume measures for CDH fetuses., Methods and Analytics: Patients with isolated CDH from singleton pregnancies will be enrolled, whose prenatal checks were performed at the Fetal Surgery Unit of the Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico (Milan, Italy) from the 30th week of gestation. A retrospective data collection of clinical and radiological variables from newborns' and mothers' clinical records will be performed for eligible patients born between 01/01/2012 and 31/12/2020. The native sequences from fetal magnetic resonance imaging (MRI) will be collected. Data from different sources will be integrated and analyzed using ML and DL, and forecasting algorithms will be developed for each outcome. Methods of data augmentation and dimensionality reduction (feature selection and extraction) will be employed to increase sample size and avoid overfitting. A software system for automatic fetal lung volume segmentation in MRI based on the DL 3D U-NET approach will also be developed., Ethics and Dissemination: This retrospective study received approval from the local ethics committee (Milan Area 2, Italy). The development of predictive models in CDH outcomes will provide a key contribution in disease prediction, early targeted interventions, and personalized management, with an overall improvement in care quality, resource allocation, healthcare, and family savings. Our findings will be validated in a future prospective multicenter cohort study., Registration: The study was registered at ClinicalTrials.gov with the identifier NCT04609163., Competing Interests: The authors have declared that no competing interests exist.

Rationale and Objectives: Acute and chronic graft rejection remains the major problem in clinical surveillance of lung-transplanted patients and early detection of complications is of capital importance to allow the optimal therapeutic option. The aim of this study was to investigate the role of quantitative non contrast-enhanced magnetic resonance imaging (MRI) as a non-ionizing imaging modality to assess ventilation impairment in patients who have undergone lung transplantation, in comparison with quantitative computed tomography (CT) and spirometry., Materials and Methods: Ten lung-transplanted patients (39 ±12 years, forced-expiratory volume in 1 second (FEV1) = 81 ± 27%, forced vital capacity (FVC) = 87 ± 27%) were acquired in breath-hold at full-expiration and full-inspiration with 1.5T MRI and CT. Maps of expiratory-inspiratory difference in MR signal-intensity and CT-density were computed to estimate regional ventilation. Based on expiratory, inspiratory, and expiratory-inspiratory difference values, each pixel was classified as healthy (H), low ventilation (LV), air trapping (AT), and consolidation (C) and the percent extent of each class was quantified., Results: Overall, expiratory-inspiratory difference in MR signal-intensity correlated to CT-density (r = 0.64, p < 0.0001) and to FEV1 (ρ = 0.71, p = 0.02). The linear correlation between MRI and CT functional maps considering all the four classes is r = 0.93 (p < 0.0001). MRI percent volumes of H, AT, and C correlated to FEV1 %pred, with the highest correlation reported for AT (ρ = -0.82)., Conclusion: Results demonstrated a good agreement between MRI and CT ventilation imaging and between the corresponding percent volumes of lung damage. Quantitative MRI may represent an accurate non-ionizing imaging technique for longitudinal monitoring of lung transplant recipients., (Copyright © 2020 The Association of University Radiologists. Published by Elsevier Inc. All rights reserved.)

Background: Panton-Valentine leukocidin (PVL) is one of the major virulence factor of Staphylococcus aureus (SA) that might be associated with invasive life-threating infections. A prompt diagnosis and adequate treatment are essential in achieving the best outcome and avoiding serious sequelae. We describe a case of severe invasive PVL-SA infection in an infant. A literature review starting from 2010 was also performed in order to discuss clinical presentations, radiological findings, treatment and outcome., Case Presentation: This is a case of a 6-month-old boy who rapidly developed high fever and poor general condition. He was diagnosed as having multiple muscular abscesses, multiple foci of osteomyelitis and bloodstream infections caused by Panton-Valentine leukocidin Methicillin-resistant Staphylococcus aureus. He received intravenous antibiotics and surgical drainage of the abscess with progressive recovery., Conclusion: Our report highlights the importance of improving awareness of this severe infection, as a prompt diagnosis and adequate manage is essential in order to save life and to prevent serious complications., (© 2021. The Author(s).)

Congenital diaphragmatic hernia is a rare disease with high mortality and morbidity due to pulmonary hypoplasia and pulmonary hypertension. The aim of the study is to investigate the relationship between radiographic lung area and systolic pulmonary artery pressure (sPAP) on the first day of life, mortality, and hernia recurrence during the first year of life in infants with a congenital diaphragmatic hernia (CDH). A retrospective data collection was performed on 77 CDH newborns. Echocardiographic sPAP value, deaths, and recurrence cases were recorded. Lung area was calculated by tracing the lung's perimeter, excluding mediastinal structures, and herniated organs, on the preoperative chest X-ray performed within 24 h after birth. Logistic and linear regression analyses were performed. Deceased infants showed lower areas and higher sPAP values. One square centimeter of rising in the total, ipsilateral, and contralateral area was associated with a 22, 43, and 24% reduction in mortality risk. sPAP values showed a decreasing trend after birth, with a maximum of 1.84 mmHg reduction per unitary increment in the ipsilateral area at birth. Recurrence patients showed lower areas, with recurrence risk decreasing by 14 and 29% per unit increment of the total and ipsilateral area. In CDH patients, low lung area at birth reflects impaired lung development and defect size, being associated with increased sPAP values, mortality, and recurrence risk. Clinical Trial Registration: The manuscript is an exploratory secondary analysis of the trial registered at ClinicalTrials.gov with identifier NCT04396028., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Amodeo, Pesenti, Raffaeli, Macchini, Condò, Borzani, Persico, Fabietti, Bischetti, Colli, Ghirardello, Gangi, Colnaghi, Mosca and Cavallaro.)

Background: MRI has been suggested as a radiation-free imaging modality to investigate early structural alterations and regional functional impairment in cystic fibrosis (CF) lung disease., Purpose/hypothesis: To compare functional and morphological MRI changes over the course of the disease to changes in spirometry., Study Type: Longitudinal retrospective study., Population: Twenty patients with CF lung disease (at baseline, age = 16.5 (13.3-20.6) years, forced expiratory volume in 1 second (as % of predicted [%pred]) FEV 1 = 71 (59-87) %pred, forced expiratory flow at 25-75% of forced vital capacity FEF 25-75 = 39 (25-63) %pred., Field Strength/sequence: 1.5T / T 2 -weighted HASTE; T 2 -weighted TSE-PROPELLER; T 2 -weighted bSSFP; T 1 -weighted 3D GRE., Assessment: Nonenhanced chest MRI and spirometry were retrospectively collected over a 3-year period from the initial recruitment visit. Images acquired at end-inspiration and end-expiration were registered by software using the optical flow method to measure expiratory-inspiratory differences in MR signal-intensity (Δ 1 H-MRI). Measures of CF functional impairment were defined from Δ 1 H-MRI: Δ 1 H-MRI median, Δ 1 H-MRI quartile coefficient of variation (QCV), and percent low-signal-variation volume (LVV). MR images were also evaluated by three readers using a CF-specific scoring system., Statistical Tests: Spearman correlation analysis, Spearman rank correlation analysis, linear mixed-effect model analysis, intraclass correlation coefficient., Results: Functional imaging parameters and total morphological score correlated with all spirometric measures, as did subscores of bronchial wall thickening/bronchiectasis, mucus plugging, and consolidation. Overall, the percent change of Δ1H-MRI median correlated with the percent change of FEV 1 (ΔFEV 1 , r = 0.41, P < 0.01) and the percent change of FEF 25-75 (ΔFEF25-75%, r = 0.38, P < 0.01). The percent change of LVV correlated with ΔFEV 1 (r = -0.47, P < 0.001) and ΔFEF 25-75 (r = -0.50, P < 0.001)., Data Conclusion: These preliminary results suggest that nonenhanced multivolume MRI may provide a feasible tool to regionally map early pulmonary alterations for longitudinal evaluation of CF lung disease, without exposing the patients to ionizing radiation., Level of Evidence: 3T TECHNICAL EFFICACY STAGE: 5., (© 2020 International Society for Magnetic Resonance in Medicine.)

In newborns with congenital diaphragmatic hernia (CDH), the radiographic lung area is correlated with functional residual capacity (FRC) and represents an alternative method to estimate lung hypoplasia. In a cohort of newborn CDH survivors, we retrospectively evaluated the relationship between radiographic lung area measured on the 1st day of life and long-term respiratory function. As a secondary analysis, we compared radiographic lung areas and respiratory function between patients undergoing fetal endoscopic tracheal occlusion (FETO) and patients managed expectantly (non-FETO). Total, ipsilateral, and contralateral radiographic areas were obtained by tracing lung perimeter as delineated by the diaphragm and rib cage, excluding mediastinal structures and herniated organs. Tidal volume (V T ), respiratory rate (RR), and their Z-Scores when compared to the norm were collected from pulmonary function tests (PFTs) performed at 12 ± 6 months of age. Linear regression analyses using the absolute Z-Score values for each parameter were performed. In CDH survivors, an increase in total and ipsilateral lung area measured at birth was related to a reduction in the absolute Z-Score for V T in PFTs ( p = 0.046 and p = 0.023, respectively), indicating a trend toward an improvement in pulmonary volumes and V T normalization. Radiographic lung areas were not significantly different between FETO and non-FETO patients, suggesting a volumetric lung increase due to prenatal intervention. However, the mean Z-Score value for RR was significantly higher in the FETO group ( p < 0.001), probably due to impaired diaphragmatic motility in the most severe cases. Further analyses are necessary to better characterize the role of the radiographic pulmonary area in the prognostic evaluation of respiratory function in patients with CDH. Clinical Trial Registration: This trial was registered at ClinicalTrials.gov with the identifier NCT04396028., (Copyright © 2020 Amodeo, Raffaeli, Pesenti, Macchini, Condò, Borzani, Persico, Fabietti, Ophorst, Ghirardello, Gangi, Colnaghi, Mosca and Cavallaro.)

Introduction:  Consensus on the best postnatal radiological evaluation of congenital lung malformations (CLMs) is still lacking. In recent years, the interest on magnetic resonance imaging (MRI) has grown, but its role is still unknown., Aim:  The aim of the study was to identify the best preoperative diagnostic assessment for CLM., Materials and Methods:  All patients with a prenatal suspicion of CLM between January 2014 and February 2018 were studied. Asymptomatic newborns underwent MRI, during spontaneous sleep without contrast. Patients with a positive MRI were scheduled for computed tomography (CT) within the fourth month of life. Thoracoscopic resection was performed in cases with a pathological CT. MRI, CT, and surgical findings were compared based on dimension, localization, and features of the CLM using the Cohen's kappa test (K)., Results:  A total of 20 patients were included (10 males). No difference was found in the diameter and site of the lesions always localized in the same side (K = 1) and in the same pulmonary lobe (K = 1). Infants who underwent thoracoscopic resection included: three congenital pulmonary airway malformations (CPAMs), five extralobar and eight intralobar sequestrations (bronchopulmonary sequestrations [BPSs]), three bronchogenic cysts, and one congenital emphysema. The concordance between MRI and CT and between radiological investigations and pathology was satisfactory for the greatest part of the studied variables. MRI showed sensitivity of 100%, specificity of 82%, positive predictive value of 50% and negative predictive value of 100% for CPAM and 77, 100, 100, and 80% for BPS, respectively., Conclusion:  MRI proved to be a reliable diagnostic investigation for CLM with high sensitivity and specificity. Early MRI in spontaneous sleep without contrast and preoperative contrast CT scan is a valuable preoperatory assessment., Competing Interests: None declared., (Thieme. All rights reserved.)

Background: Acute otitis media is one of the most common infectious diseases in the paediatric age and although its complications such as acute mastoiditis have become rare thanks to improvements in therapeutic approaches, possible serious complications such as septic arthritis of the temporomandibular joint may develop. A prompt diagnosis and adequate treatment are essential to achieving the best outcome and avoiding serious sequelae. We describe a case occurring in a previously healthy 6-year-old female and review the literature currently available on this topic., Case Presentation: The patient presented a right temporomandibular septic arthritis with initial mandibular bone involvement secondary to acute otitis media. She presented with torcicollis, trismus, right preauricular swelling over the temporomandibular joint and was successfully treated with antibiotic treatment alone., Conclusions: Septic arthritis of the temporomandibular joint is a rare complication of acute otitis media or acute mastoiditis in children. It should be suspected in patients presenting with trismus, preauricular swelling or fever. No guidelines on the diagnosis and treatment of this infectious disease are currently available.

This cross-sectional study aims to verify the relationship between quantitative multivolume proton-magnetic resonance imaging ( 1 H-MRI) and clinical indicators of ventilatory abnormalities in cystic fibrosis (CF) lung disease.Non-enhanced chest MRI, spirometry and multiple breath washout was performed by 28 patients (10-27 years) with CF lung disease. Images acquired at end-inspiration and end-expiration were registered by optical flow to estimate expiratory-inspiratory proton-density change (Δ 1 H-MRI) as a measure of regional ventilation. Magnetic resonance images were also evaluated using a CF-specific scoring system.Biomarkers of CF ventilation impairment were defined from the Δ 1 H-MRI as follows: Δ 1 H-MRI median, Δ 1 H-MRI quartile coefficient of variation (QCV) and percentage of low-ventilation volume (%LVV). Imaging biomarkers correlated to all the clinical measures of ventilation abnormality, with the strongest correlation between Δ 1 H-MRI median and forced expiratory volume in 1 s (r 2 =0.44, p<0.001), Δ 1 H-MRI QCV and lung clearance index (LCI) (r 2 =0.51, p<0.001) and %LVV and LCI (r 2 =0.66, p<0.001). Correlations were also found between imaging biomarkers of ventilation and morphological scoring.The study showed a significant correlation between quantitative multivolume MRI and clinical indicators of CF lung disease. MRI, as a non-ionising imaging technique, may be particularly attractive in CF care for longitudinal evaluation, providing a new imaging biomarker to detect early ventilatory abnormalities., Competing Interests: Conflict of interest: F. Pennati has nothing to disclose. Conflict of interest: C. Salito has nothing to disclose. Conflict of interest: I. Borzani has nothing to disclose. Conflict of interest: G. Cervellin has nothing to disclose. Conflict of interest: S. Gambazza reports fees for consulting on lung clearance index from Vertex Pharmaceuticals. Conflict of interest: R. Guarise has nothing to disclose. Conflict of interest: M.C. Russo has nothing to disclose. Conflict of interest: C. Colombo has nothing to disclose. Conflict of interest: A. Aliverti has nothing to disclose., (Copyright ©ERS 2019.)

Aim: To evaluate tracheal diameters and their clinical impact in patients with congenital diaphragmatic hernia (CDH) after fetal endoscopic tracheal occlusion (FETO)., Methods: Patients born with CDH between January 2012 and August 2016 were divided into two groups: noFETO and FETO. Tracheal diameters at three levels (T1, carina, and maximum tracheal dilation) on chest X-ray at 1, 3, 6, 12, 24, and 36 months of follow-up, requirements of invasive and noninvasive respiratory support, the incidence of respiratory infections, and results of pulmonary function tests (PFT) were compared., Results: A total of 71 patients with CDH were born in the study period, and there were 34/41 survivors in the no-FETO group (82.9%) and 13/30 in the FETO group (43.3%). The maximum tracheal diameter was significantly greater in the FETO group at all ages. No differences were observed in the diameters at T1 and the carina, in the requirements of invasive and noninvasive respiratory support, and in the incidence respiratory infections. At the PFT (6-12 months), the FETO group presented higher respiratory rates (46.1 ± 6.2 vs. 36.5 ± 10.6, p = 0.02). No differences in PFT results were found between the groups after the 1st year of life., Conclusions: The FETO procedure leads to persistent tracheomegaly. However, the tracheomegaly does not seem to have a significant clinical impact., (© 2018 S. Karger AG, Basel.)

Background: Double gallbladder is a rare biliary anomaly. Perinatal diagnosis of the disorder has been reported in only 6 cases, and in 5 of them the diagnosis was based on ultrasound imaging only. However, the ultrasound technique alone does not provide a sufficiently precise description of cystic ducts and biliary anatomy, an information that is crucial for a correct classification and for a possible future surgery., Case Presentation: At 21 weeks of gestational age of an uneventful pregnancy in a 38 year old primipara mother, a routine ultrasound screening detected a biliary anomaly in the fetus suggestive of a double gallbladder. A neonatal abdominal ultrasonography performed on postnatal day 2 confirmed the diagnosis. On day 12 the newborn underwent a Magnetic Resonance Cholangiopancreatography (MRCP) that clearly characterized the anatomy of the anomaly: both gallbladders had their own cystic duct and both had a separate insertion in the main biliary duct., Conclusions: We report a case of early prenatal suspected duplicate gallbladder that was confirmed by a neonatal precise diagnosis of a Type 2, H or ductular duplicate gallbladder, using for the first time 3D images of Magnetic resonance cholangiopancreatography in a newborn. An accurate anatomical diagnosis is mandatory in patients undergoing a possible future cholecystectomy, to avoid surgical complications or reoperations. Therefore, in case of a perinatal suspicion of a double gallbladder, neonates should undergo a Magnetic resonance cholangiopancreatography. A review of the Literature about this variant is included.

Rationale: Lymphatic malformations (LMs) are rare and benign anomalies resulting from the defective embryological development of the primordial lymphatic structures. Due to their permeative growth throughout all tissue layers, treatment is often challenging. Small asymptomatic lesions can be conservatively managed, while symptomatic lesions require active management. Surgery has been historically considered the treatment of choice, but today less invasive therapeutic options are preferred (sclerotherapy, laser therapy, oral medications). However, there are not uniform therapeutic protocols. Sirolimus is an oral medication that has been reported to be effective in the recent literature. Here we present the case of 4 newborns with giant multicystic lymphangioma treated with oral sirolimus after surgical resection had failed., Patient Concerns: At birth the LMs were clinically appreciated as giant masses involving different organs and structures., Diagnoses: All patients had a prenatal diagnosis of giant multicystic lymphangioma confirmed at histological and cytological analysis., Interventions: Patients were treated with oral sirolimus after unsuccessful surgical resection., Outcomes: In all patients, sirolimus determined an overall reduction of the mass and a global involution from the macro- to the microcystic composition. Sirolimus was safe and poor disadvantages had been observed. The main and isolated adverse effect at laboratory analysis was progressive dyslipidemia, with increasing levels of total cholesterol and triglycerides., Lessons: To date, our experience with sirolimus in the management of LMs is favorable. We recommend the use of sirolimus after unsuccessful surgical excision have been tried or when the surgical approach is not feasible. A multidisciplinary follow-up is needed to monitor disease evolution., (Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.)

Background/purpose: To assess the anatomical results after posterior sagittal anorectoplasty (PSARP) by magnetic resonance imaging (MRI) and to evaluate the correlation of these findings with clinical outcomes., Methods: Patients followed-up at our center after PSARP, being at least 6year old, with neither evidence of sacral abnormalities nor spinal dysraphisms were prospectively included. Complex ARMs were excluded. MRI was performed on a 1.5T unit. T1- and T2-weighted sequences were acquired, in orthogonal planes, according to the anal canal orientation. The degree of anorectal centering in the muscle complex (DARC), the pelvic floor symmetry, the rectal maximum diameter, the fat tissue interposition, and the presence of fibrosis were evaluated. A clinical questionnaire (Rintala score) was filled in by the parents. MRI findings were compared to the clinical outcomes assessed by the Rintala score. For statistical analysis the Spearman rho correlation coefficient was calculated and the Wilcoxon rank-sum test was performed., Results: We recruited 11 patients (mean age 12years, range 6-19) with MRI. DARC (range 252-360°) was strongly correlated with the degree of fecal incontinence (rho=0.70), mildly with the ability to hold back defecation (rho=0.58), constipation (rho=0.46) and total Rintala score (rho=0.41). Pelvic floor symmetry correlated with the frequency of defecation (rho=0.58). Rectal maximum diameter negatively correlated with the ability to feel the urge to defecate (rho=-0.60). Patients with fibrosis were slightly more constipated (p=0.056) and presented more social impairment (p=0.04). Fat tissue interposition had no correlation with the clinical outcome., Conclusions: Thanks to soft tissue definition, multiplanar imaging, and lack of ionizing radiation, MRI is a valuable tool in the postoperative anatomical evaluation of patients with ARM. Our preliminary results show that abnormal anatomical findings can correlate to a nonoptimal functional outcome, thus helping in understanding the clinical course. The degree of anorectal centering in the muscle complex (DARC) seems to have the better correlation with the outcome, especially in terms of fecal continence., Type of Study: Prognosis study., Level of Evidence: level IV., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Rib osteomyelitis is a rare entity, occurring in approximately 1 % or less of all cases of haematogenous osteomyelitis. Given its rarity and clinical heterogeneity, the diagnosis of rib osteomyelitis can be challenging and requires a high index of suspicion. We present a case of acute osteomyelitis of the rib due to community-acquired methicillin-resistant Staphylococcus aureus (MRSA), which occurred in an otherwise healthy 3-month-old infant and mimicked an epigastric hernia at first., Case Presentation: An otherwise healthy 3-month-old female infant was sent by her primary care paediatrician to the paediatric emergency department for possible incarcerated epigastric hernia because for 2 days, she had suffered from mild to moderate fever, irritability, poor feeding, and tender epigastric swelling. Ultrasonographic imaging excluded epigastric hernia, and transthoracic echocardiography ruled out endocarditis. However, clinical assessment combined with laboratory criteria classified the child into the high-risk group for having severe bacterial infection. Consequently, awaiting the definitive diagnosis, she was immediately treated with a broad-spectrum regimen of intravenous antibiotic therapy based on vancomycin (40 mg/kg/die in 3 doses) and meropenem (100 mg/kg/die in 3 doses). Three days after admission, the blood culture result was positive for methicillin-resistant Staphylococcus aureus, and vancomycin remained as antibiotic therapy. On day 3, a second swelling appeared at the level of the seventh left rib, 2 cm-wide, non-erythematous, mildly painful. Ultrasonography of the left chest wall on this occasion showed an image consistent with an acute osteomyelitis of the anterior osteo-chondral region of the 7th rib and associated adjacent periosteal and soft tissue collection and magnetic resonance imaging confirmed the osteomyelitis of the anterior middle-distal part of the 7th left rib, near the costochondral junction. Vancomycin was continued up to a total of 6 weeks of therapy, and at the end, the child was discharged in good condition with no relapse during the follow-up., Conclusion: This is one of the few reported cases of paediatric rib osteomyelitis caused by community-acquired MRSA. Timely identification associated with prompt and targeted antibiotic therapy may allow full recovery.

The relation of infectious agents to arthritis is an area of great interest to the rheumatologist. Septic arthritis of bacterial origin accounts for approximately 6.5% of all childhood arthritides. Septic arthritis usually results from haematogenous spread from a focus of infection elsewhere in the body, but also by direct extension of an infection from overlying soft tissues or bone or traumatic invasion of the joint. As a result, if a focus of underlying osteomyelitis breaks throught the metaphysis, it may enter the joint and result in septic arthritis. Systemic signs of illness are fever, severe bone pain, and tenderness with or without local swelling. A wide range of microorganism can cause septic arthritis in children; Staphylococcus aureus and nongroup A and B streptococci are most common overall. However, different organisms are more common at some ages and in certain circumstances. Kingella kingae is an emerging pathogen in young children under 4 years of age. The clinical presentation of K. kingae invasive infection is often subtle and may be associated to mild to moderate biologic inflammatory responses. Affected children often have few signs and symptoms of osteoarticular infections. Early MRI is useful in differentiating K kingae from Gram-positive cocci in osteoarticular infections. Cartilaginous involvement, modest soft tissue and bone reaction suggest K. kingae. It's very important to include K. kingae in differential diagnosis of osteoarticular infections in young children. We report an unusual case of osteomyelitis: clinical manifestations and MRI are suggestive for K kingae infection.

Background: There are few prospective evaluations of point-of-care ultrasonography (US) for the diagnosis of pediatric community-acquired pneumonia (CAP). In particular, there are very few data concerning the efficiency of US in comparison with that of chest radiography (CR) in defining different kinds of lung alterations in the various pulmonary sections. The aim of this study was to bridge this gap in order to increase our knowledge of the performance of US in diagnosing CAP in childhood., Methods: A total of 103 children (56 males, 54.4%; mean age ± standard deviation 5.6 ± 4.6 years) admitted to hospital with a clinical diagnosis of suspected CAP were prospectively enrolled and underwent CR (evaluated by an independent expert radiologist) and lung US (performed by a resident in paediatrics with limited experience in US). The performance of US in diagnosing CAP (i.e. its sensitivity, specificity, and positive and negative predictive values) was compared with that of CR., Results: A total of 48 patients had radiographically confirmed CAP. The sensitivity, specificity, and positive and negative predictive values of US in comparison with CR were respectively 97.9%, 94.5%, 94.0% and 98.1%. US identified a significantly higher number of cases of pleural effusion, but the concordance of the two methods in identifying the type of CAP was poor., Conclusion: US can be considered a useful means of diagnosing CAP in children admitted to an Emergency Department with a lower respiratory tract infection, although its usefulness in identifying the type of lung involvement requires further evaluation.

Background: Little is known about the prevalence of viral infections in children with community-acquired pneumonia (CAP)., Objectives: To describe the clinical and virological data collected from children with radiographically confirmed CAP in whom 17 respiratory viruses were sought in respiratory secretion samples during the acute phase of the disease., Patients and Methods: The study involved 592 children with radiographically confirmed CAP whose respiratory secretion samples were tested using the Luminex xTAG Respiratory Virus Panel Fast assay, which simultaneously detects influenza A virus, influenza B virus, respiratory syncytial virus (RSV)-A and -B, parainfluenzavirus-1, -2, -3, and -4, adenovirus, human metapneumovirus, coronaviruses 229E, NL63, OC43, and HKU1, enterovirus/rhinovirus, and bocavirus. A real-time PCR assay was used to identify the rhinovirus in the enterovirus/rhinovirus-positive samples., Results: A total of 435 children (73·5%) were positive for at least one virus: the most frequently detected was RSV, which was found in 188 (31·7%), followed by rhinovirus (n = 144, 24·3%), bocavirus (n = 60, 10·1%), influenza viruses (n = 57, 9·6), and hMPV (n = 49, 8·2%). Viral co-infections were found in 117 children (19·7% of the enrolled children; 26·9% of those with viral infections). Marginal differences were found between the infections owing to a single virus. Co-infections showed radiographic evidence of alveolar pneumonia significantly more frequently than single infections (OR 1·72, 95% CI 1·05-2·81)., Conclusions: The findings of this study highlight the importance of respiratory viruses (mainly RSV and rhinovirus) in children with CAP and show the characteristics of both the single infections and co-infections associated with the disease., (© 2012 Blackwell Publishing Ltd.)

This study of 592 children seen in our Emergency Department with radiographically confirmed community-acquired pneumonia (CAP) was designed to evaluate the role of rhinoviruses (RVs) in the disease. The respiratory secretions of each child were assayed using RVP Fast in order to detect 17 respiratory viruses, and the RV-positive samples were characterised by means of real-time polymerase chain reaction and sequencing. RVs were identified in 172 cases (29.0%): 48/132 children aged<1 year (36.3%), 80/293 aged 1-3 years (27.3%), and 44/167 aged≥4 years (26.3%). Sequencing demonstrated that 82 RVs (49.1%) were group A, 17 (10.1%) group B, and 52 (31.1%) group C; 21 (12.2%) were untyped. RVs were found as single agents in 99 cases, and together with two or more other viruses in 73 (40.7%). There were only marginal differences between the different RV groups and between single RV infection and RV co-infections. RV CAP is frequent not only in younger but also in older children, and RV-A is the most common strain associated with it. The clinical relevance of RV CAP seems to be mild to moderate without any major differences between the A and B strains and the recently identified RV C.

Noninflammatory tubal abnormalities are rare in children and usually not well covered by traditional educational material. The presenting symptoms are nonspecific and are common to many other conditions, so its preoperative diagnosis is rarely made. The purpose of this study was to review the hospital charts and imaging findings in children and sexually inactive adolescents who showed fallopian tube pathology. Understanding of the pertinent findings of previous imaging examinations might assist radiologists in making the correct preoperative diagnosis and increase the likelihood of preserving the fallopian tubes. The clinical entities described in this article include isolated tubal torsion, paratubal cysts, hydrosalpinx, undescended/ectopic fallopian tube, and tubal inguinal hernia.

Objective: To verify the earliest detectability of liver metastases in patients who underwent serial multidetector computed tomography (MDCT) examinations., Methods: We selected 12 patients with known primary cancer who underwent 4 or more contrast-enhanced, 4-detector MDCTs. When metastases had been reported, an evaluation of the preceding MDCT was done to define whether the lesion was detectable, detectable only by minimal signs, undetectable, or detected but misdiagnosed as a benign lesion (MBL)., Results: Eighty-eight lesions were analyzed. Evaluating the preceding examination, we defined detectable (n=8), detectable only by minimal signs (n=5), undetectable (n=74), and MBL (n=1). The group with minimal signs was composed of 4 small hypodense foci and 1 calcification. The MBL was a non-Hodgkin lesion first misdiagnosed as a hemangioma., Conclusion: Approximately 15% of liver metastases were prospectively missed, 9% of them being retrospectively detectable, 6% being retrospectively visible as minimal signs, whereas only 1% of liver metastases were misdiagnosed as a benign lesion.