The role of point-of-care ultrasound in the management of neonates with congenital diaphragmatic hernia.

In the last few years, current evidence has supported the use of point-of-care ultrasound (POCUS) for a number of diagnostic and procedural applications. Considering the valuable information that POCUS can give, we propose a standardized protocol for the management of neonates with a congenital diaphragmatic hernia (CDH-POCUS protocol) in the neonatal intensive care unit. Indeed, POCUS could be a valid tool for the neonatologist through the evaluation of 1) cardiac function and pulmonary hypertension; 2) lung volumes, postoperative pleural effusion or pneumothorax; 3) splanchnic and renal perfusion, malrotations, and/or signs of necrotizing enterocolitis; 4) cerebral perfusion and eventual brain lesions that could contribute to neurodevelopmental impairment. In this article, we discuss the state-of-the-art in neonatal POCUS for which concerns congenital diaphragmatic hernia (CDH), and we provide suggestions to improve its use. IMPACT: This review shows how point-of-care ultrasound (POCUS) could be a valid tool for managing neonates with congenital diaphragmatic hernia (CDH) after birth. Our manuscript underscores the importance of standardized protocols in neonates with CDH. Beyond the well-known role of echocardiography, ultrasound of lungs, splanchnic organs, and brain can be useful. The use of POCUS should be encouraged to improve ventilation strategies, systemic perfusion, and enteral feeding, and to intercept any early signs related to future neurodevelopmental impairment.

A child with polyarthritis and chronic lung disease: a case report of ataxia-telangiectasia.

BACKGROUND: Ataxia-telangiectasia (A-T) is a rare autosomal recessive DNA repair disorder, characterized by progressive cerebellar degeneration, telangiectasia, immunodeficiency, recurrent sinopulmonary infections, radiation sensitivity, premature aging and predisposition to cancer. Although the association with autoimmune and chronic inflammatory conditions such as vitiligo, thrombocytopenia and arthritis has occasionally been reported, an onset with articular involvement at presentation is rare. CASE PRESENTATION: We herein report the case of a 7-year-old Caucasian girl who was admitted to the Rheumatology Department with a history of febrile chough and polyarthritis which led initially to the suspicion of an autoinflammatory disease. She had overt polyarthritis with knees deformities and presented with severe pneumonia. A chest Computed Tomography (CT) scan showed bilateral bronchiectasis, parenchymal consolidation and interstitial lung disease; rheumatoid factor and type I interferon signature resulted negative, therefore excluding COatomer Protein subunit Alpha (COPA) syndrome. A diagnosis of sarcoidosis had been suspected based on histological evidence of granulomatous liver inflammation, but ruled out after detecting normal angiotensin converting enzyme and chitotriosidase blood levels. Based on her past medical history characterized by at least six episodes of pneumonia in the previous 4 years, immunological phenotyping was performed. This showed complete IgA and IgE deficiency with defective antigen-specific antibodies to Pneumococcal, Tetanus toxin and Hemophilus Influenzae B vaccines. Additionally, low numbers of B cells and recent thymic emigrants (RTE) were found (CD4Ra 1.4%), along with a low CD4+/CD8 + T cells ratio (< 1). Finally, based on gait disturbances (wobbly wide-based walking), serum alfa-fetoprotein was dosed, which resulted increased at 276 ng/ml (normal value < 7 ng/ml). A diagnosis of Ataxia-Telangiectasia was made, strengthened by the presence of bulbar telangiectasia, and then confirmed by Whole Exome Sequencing (WES). CONCLUSIONS: Although rare, A-T should always be ruled out in case of pulmonary bronchiectasis and gait disturbances even in the absence of bulbar or skin telangiectasia. Autoimmune and granulomatous disorders must to be considered as differential diagnosis.

Retrocardiac Pneumomediastinum: Description of an Unusual Case and Review of Literature.

Retrocardiac pneumomediastinum (RP) is the consequence of air trapping in the inferior and posterior mediastinum. It is characterized by the presence of a right or left para-sagittal infrahilar oval or pyramidal air collection on a chest X-ray. It is usually detected in neonates because of alveolar rupture after invasive ventilation or invasive manoeuvres applied on airways or the digestive tract. A healthy child came to the emergency department (ED) for acute respiratory failure due to viral bronchiolitis when he was 2 months old. Because of his clinical condition, he underwent helmet continuous positive airway pressure (HCPAP). When the condition allowed, he was discharged and sent home. He was re-admitted into the hospital for asthmatic bronchitis 3 months later. A frontal chest X-ray taken during the second hospitalization showed an oval-shaped retrocardiac air lucency not previously detected. Differential diagnosis including digestive and lung malformations was made. Finally, the diagnosis of RP was made. We report an unusual case of retrocardiac pneumomediastinum in a 5-month-old male infant after the application of continuous positive pressure via a helmet. RP presentation after the administration of non-invasive ventilatory support beyond the neonatal age is unusual. Although surgical drainage is curative, conservative treatment can be considered in hemodynamically stable patients.

Communicating radiation dose in medical imaging: How to best inform our patients?

Background: The newly adopted European directive DE59/2013 mandates adequate patient information in procedures involving ionising radiation. Patient interest in knowing about their radiation dose and an effective communication method for dose exposure remain poorly investigated. Purpose: This study is aimed at investigating both patient interest in radiation dose and an effective method to communicate radiation dose exposure. Material and methods: The present analysis is based on a multi-centre cross-sectional data collection involving 1,084 patients from four different hospitals ‒ two general and two paediatric hospitals. Anonymous questionnaires were administered, consisting of an initial overview of radiation use in imaging procedures, a patient data section, and an explanatory section providing information in four modalities. Results: 1009 patients were included in the analysis, with 75 refusing participation; 173 participants were relatives of paediatric patients. Initial information provided to patients was considered comprehensible. The information modality with symbols was considered the most readily understandable format by patients, with no appreciable differences in comprehension attributable to social or cultural background. The modality including dose numbers and diagnostic reference levels was preferred by patients with higher socio-economic background. The option 'None of those' was selected by one-third of our sample population, composed of four different clusters: female, over 60 years old, unemployed, and from low socio-economic backgrounds. Conclusions: This study demonstrated a high level of interest amongst patients in knowing about radiation dose exposure. Pictorial representations were well understood by patients from a variety of different ages and education levels. However, a universally comprehensible model of communicating radiation dose information remains to be elucidated.

The effect of selective ultrasound screening on the incidence of late presentation of developmental hip dysplasia-a meta-analysis.

Different screening strategies for developmental dysplasia of the hip (DDH) exist. Despite screening efforts, cases of late presentation continue to occur, often necessitating surgery. This systematic review and meta-analysis assess the effect of newborn selective ultrasound screening for DDH on the incidence of late presentation in infants and children, compared to a universal ultrasound strategy. A systematic search across Medline and EMBASE databases was performed between January 1950 and February 2021. A consensus-based evaluation of abstracts led to retrieval of relevant full text, original articles or systematic reviews in English only. These were assessed according to agreed eligibility criteria, and their reference lists were reviewed to identify additional eligible publications. Following final consensus on included publications, data was extracted, analysed and reported as per PRISMA and Prospero (CRD42021241957) guidelines. The 16 eligible studies consisted of 2 randomised controlled trials and 14 cohort studies, published between 1989 and 2014, with a total of 511,403 participants. In total, 121,470 (23.8%) received a neonatal hip ultrasound, of whom 58,086 and 63,384 were part of a selective or a universal ultrasound screening strategy, respectively. The difference in the proportion of late presentation between the universal and selective strategies was 0.0904 per 1,000 (P = 0.047). The time effect, i.e. the difference between early and late presentation defined respectively, as less than and more than 3 months of age, regardless of screening strategy, was not significant (P = 0.272). Although there was variability in study design and reporting, the quality of the evidence, based on the critical appraisal skills programme appraisal tools, was generally good. Compared to universal ultrasound screening for DDH, selective screening resulted in a slightly higher rate of late presentation. Uniformity in design and reporting of DDH studies and a cost-effectiveness analysis are needed.

Safety and Efficacy of Mek Inhibitors in the Treatment of Plexiform Neurofibromas: A Retrospective Study.

INTRODUCTION: Plexiform neurofibromas (PN) represent the main cause of morbidity in patients affected by Neurofibromatosis Type 1 (NF1). Until recently, surgery has been the main treatment option in these patients, but it is burdened with a low efficacy rate and a high incidence of side effects as well as recurrence. In recent years, MEK inhibitors (MEKi) such as selumetinib and trametinib have shown great promise. METHODS: We retrospectively describe a single center cohort of NF1 patients affected by PN1 and treated with MEKi since 2019 to 2021. Patients recruited in the study were affected by PN that were not eligible to complete surgical excision, symptomatic or with major cosmetic deformation or functional neurological deficits. RESULTS: Most patients experienced improvement in clinical symptoms and quality of life, with reduction or stabilization of lesions. However, no complete response was achieved. The most common adverse effects involved the skin, affecting every patient. Importantly, no life-threatening adverse effects occurred. CONCLUSIONS: In our experience, MEKi treatment has been shown to be both safe and effective in improving symptomatology and quality of life.

Hepatic hemangiomas in childhood: the spectrum of radiologic findings. A pictorial essay.

Hepatic hemangiomas (HHs) are the most common benign liver tumors in infancy, but despite their frequent occurrence, their radiologic appearance may be particularly challenging to assess, owing to unique anatomical and development features that distinguish their subtypes in the pediatric population. There are 3 main patterns of the lesions recognized in HHs: focal, multifocal and diffuse. Medical care strategies range from simple observation to surgery, and a timely diagnosis is crucial to establish the most appropriated clinical management and therapy. Ultrasound (US) is typically the first level examination of pediatric vascular anomalies, able to meet a satisfactory diagnosis and has proved very helpful to assess for involution through serial imaging. CT and MRI are second-level methods seldom performed, offering an anatomical panoramic view and clarification when US is non-conclusive. Histologic confirmation is rarely required. HHs show a variable sonographic appearance, with hypoechoic (mostly) or hyperechoic solitary\multifocal mass and wide features. Hepatic vessels assessment reveals an abnormal flow, enlarged vasa, decreased resistive index (RI) values, scarce blood supply distally to lesions and hyper vascular regions inside them. In this pictorial essay, we review HHs subtypes in further details, illustrate US, CT and MRI findings and clarify a recurrent dispute over the existing terminology.

A novel magnetic resonance imaging scoring system for active and chronic changes in children and adolescents with juvenile idiopathic arthritis of the hip.

BACKGROUND: Hip involvement predicts severe disease in juvenile idiopathic arthritis (JIA) and is accurately assessed by MRI. However, a child-specific hip MRI scoring system has not been validated. OBJECTIVE: To test the intra- and interobserver agreement of several MRI markers for active and chronic hip changes in children and young adults with JIA and to examine the precision of measurements commonly used for the assessment of growth abnormalities. MATERIALS AND METHODS: Hip MRIs from 60 consecutive children, adolescents and young adults with JIA were scored independently by two sets of radiologists. One set scored the same MRIs twice. Features of active and chronic changes, growth abnormalities and secondary post-inflammatory changes were scored. We used kappa statistics to analyze inter- and intraobserver agreement for categorical variables and a Bland-Altman approach to test the precision of continuous variables. RESULTS: Among active changes, there was good intra- and interobserver agreement for grading overall inflammation (kappa 0.6-0.7). Synovial enhancement showed a good intraobserver agreement (kappa 0.7-0.8), while the interobserver agreement was moderate (kappa 0.4-0.5). Regarding acetabular erosions on a 0-3 scale, the intraobserver agreement was 0.6 for the right hip and 0.7 for the left hip, while the interobserver agreement was 0.6 for both hips. Measurements of joint space width, caput-collum-diaphyseal angle, femoral neck-head length, femoral width and trochanteric distance were imprecise. CONCLUSION: We identified a set of MRI markers for active and chronic changes in JIA and suggest that the more robust markers be included in future studies addressing clinical validity and long-term patient outcomes.

Fibrolipomatous hamartoma of the median nerve in a child with carpal tunnel syndrome: imaging findings and literature review.

Fibrolipomatous hamartoma of the median nerve is an uncommon benign tumour of the childhood, which usually manifests in adolescents or adulthood with signs of compressive neuropathy at wrist. Symptomatic tumour is unusual in children below 5 years age and can be underdiagnosed. Magnetic resonance imaging provides pathognomonic features for the diagnosis, obviating the need for biopsy. Although standard ultrasonography is frequently the first-line imaging approach in the evaluation of soft-tissue masses, sonographic findings of this lesion are less frequently reported and have to be kept in mind by radiologist. We report the unusual case of carpal tunnel syndrome secondary to fibrolipomatous hamartoma of the median nerve in a 4-year-old child successfully treated with surgical carpal tunnel release.

Machine Learning Algorithm: Texture Analysis in CNO and Application in Distinguishing CNO and Bone Marrow Growth-Related Changes on Whole-Body MRI.

OBJECTIVE: The purpose of this study is to analyze the texture characteristics of chronic non-bacterial osteomyelitis (CNO) bone lesions, identified as areas of altered signal intensity on short tau inversion recovery (STIR) sequences, and to distinguish them from bone marrow growth-related changes through Machine Learning (ML) and Deep Learning (DL) analysis. MATERIALS AND METHODS: We included a group of 66 patients with confirmed diagnosis of CNO and a group of 28 patients with suspected extra-skeletal systemic disease. All examinations were performed on a 1.5 T MRI scanner. Using the opensource 3D Slicer software version 4.10.2, the ROIs on CNO lesions and on the red bone marrow were sampled. Texture analysis (TA) was carried out using Pyradiomics. We applied an optimization search grid algorithm on nine classic ML classifiers and a Deep Learning (DL) Neural Network (NN). The model's performance was evaluated using Accuracy (ACC), AUC-ROC curves, F1-score, Positive Predictive Value (PPV), Mean Absolute Error (MAE) and Root-Mean-Square Error (RMSE). Furthermore, we used Shapley additive explanations to gain insight into the behavior of the prediction model. RESULTS: Most predictive characteristics were selected by Boruta algorithm for each combination of ROI sequences for the characterization and classification of the two types of signal hyperintensity. The overall best classification result was obtained by the NN with ACC = 0.91, AUC = 0.93 with 95% CI 0.91-0.94, F1-score = 0.94 and PPV = 93.8%. Between classic ML methods, ensemble learners showed high model performance; specifically, the best-performing classifier was the Stack (ST) with ACC = 0.85, AUC = 0.81 with 95% CI 0.8-0.84, F1-score = 0.9, PPV = 90%. CONCLUSIONS: Our results show the potential of ML methods in discerning edema-like lesions, in particular by distinguishing CNO lesions from hematopoietic bone marrow changes in a pediatric population. The Neural Network showed the overall best results, while a Stacking classifier, based on Gradient Boosting and Random Forest as principal estimators and Logistic Regressor as final estimator, achieved the best results between the other ML methods.

Vascular lesions of the pediatric orbit: A radiological walkthrough.

Vascular anomalies of the pediatric orbit represent a heterogeneous group that include both vascular tumors and vascular malformations. The disorder may initially be silent and then associated with symptoms and/or function damage, depending on the type of vascular anomaly and its extension. Vascular tumors include benign, locally aggressive (or borderline) and malignant forms while vascular malformations are divided into "simple", "combined" and syndromic, or "low flow" or "high flow". Both entities can arise in isolation or as part of syndromes. In this review, we describe the imaging findings of the vascular lesions of the orbit in the pediatric population, which are key to obtain a correct diagnosis and to guide the appropriate treatment in the light of the new genetic and molecular discoveries, and the role of the radiologist in their multidisciplinary management. We will also touch upon the main syndromes associated with orbital vascular abnormalities.

Central Nervous System Metastasis in Neuroblastoma: From Three Decades Clinical Experience to New Considerations in the Immunotherapy Era.

Central nervous system (CNS) metastatic spread in neuroblastoma (NB) is rare and occurs more often at relapse/progression. We report on CNS involvement in high risk (HR) NB over 25 years. For this retrospective study, we reviewed the CNS imaging of all the patients treated at Bambino Gesù Children Hospital from 1 July 1996 to 30 June 2022. A total of 128 patients with HR NB were diagnosed over 26 years. Out of 128 patients, CNS metastatic spread occurred in 6 patients: 3 patients presented a metastatic spread at diagnosis, while in 3 patients, CNS was involved at relapse. Overall, the rate of occurrence of CNS spread is 4.7% with the same distribution at diagnosis and at relapse, namely 2.3%. Interestingly, CNS spread at diagnosis was observed only before 2012, whereas CNS was observed at relapse only after 2012, in the immunotherapy era. CNS metastases presented similar imaging features at diagnosis and at relapse, with a peculiar hemorrhagic aspect and mainly hemispheric localization in patients with bone skull involvement at the time of diagnosis. The outcome is dismal, and 3 out of 6 patients died for progressive disease.

The Pitfall of Ganglioneuroblastoma-Nodular Diagnosis: Clinical and Imaging Considerations over a Rare Bifocal Sporadic Case.

Neuroblastic tumors (NTs) represent the most common extracranial neoplasm occurring in childhood. Although ganglioneuroblastoma intermixed (GNBI) and ganglioneuroma (GN) are classified as very low-risk tumors, neuroblastoma (NB) and ganglioneuroblastoma-nodular (GNBN) may represent a serious risk to survival. Unfortunately, areas of GNBI and GNBN can coexist in the same mass, leading to incorrect risk staging when only biopsy is performed. Herein, we describe a case of multifocal NT (thoracic and abdominal localization) occurring in a 4-year-old male. Different histological subtypes, namely GNBI and GNBN, were revealed in the two lesions. We focus on the difficulties of proper diagnosis and risk stratification, underlining the usefulness of several diagnostic tools for appropriate management and therapeutic choices.

Developing a Predictive Grading Model for Children with Gliomas Based on Diffusion Kurtosis Imaging Metrics: Accuracy and Clinical Correlations with Patient Survival.

Purpose: To develop a predictive grading model based on diffusion kurtosis imaging (DKI) metrics in children affected by gliomas, and to investigate the clinical impact of the predictive model by correlating with overall survival and progression-free survival. Materials and methods: 59 patients with a histological diagnosis of glioma were retrospectively studied (33 M, 26 F, median age 7.2 years). Patients were studied on a 3T scanner with a standardized MR protocol, including conventional and DKI sequences. Mean kurtosis (MK), axial kurtosis (AK), radial kurtosis (RK), fractional anisotropy (FA), and apparent diffusion coefficient (ADC) maps were obtained. Whole tumour volumes (VOIs) were segmented semi-automatically. Mean DKI values were calculated for each metric. The quantitative values from DKI-derived metrics were used to develop a predictive grading model to develop a probability prediction of a high-grade glioma (pHGG). Three models were tested: DTI-based, DKI-based, and combined (DTI and DKI). The grading accuracy of the resulting probabilities was tested with a receiver operating characteristics (ROC) analysis for each model. In order to account for dataset imbalances between pLGG and pHGG, we applied a random synthetic minority oversampling technique (SMOTE) analysis. Lastly, the most accurate model predictions were correlated with progression-free survival (PFS) and overall survival (OS) using the Kaplan−Meier method. Results: The cohort included 46 patients with pLGG and 13 patients with pHGG. The developed model predictions yielded an AUC of 0.859 (95%CI: 0.752−0.966) for the DTI model, of 0.939 (95%CI: 0.879−1) for the DKI model, and of 0.946 (95%CI: 0.890−1) for the combined model, including input from both DTI and DKI metrics, which resulted in the most accurate model. Sample estimation with the random SMOTE analysis yielded an AUC of 0.98 on the testing set. Model predictions from the combined model were significantly correlated with PFS (25.2 months for pHGG vs. 40.0 months for pLGG, p < 0.001) and OS (28.9 months for pHGG vs. 44.9 months for pLGG, p < 0.001). Conclusions: a DKI-based predictive model was highly accurate for pediatric glioma grading. The combined model, derived from both DTI and DKI metrics, proved that DKI-based model predictions of tumour grade were significantly correlated with progression-free survival and overall survival.

E-cigarette or Vaping product use Associated Lung Injury (EVALI) in a 15 year old female patient - case report.

BACKGROUND: E-cigarettes are devices which allow to aerosolize liquids containing nicotine or other substances. Ever since they were released on the market in 2006, the number of users have been constantly increasing, especially among adolescents, ranging from 7,6% to 9,3% in the age group 18-24 years old from 2014 to 2019. Hand in hand with the spread of E-cigarettes many have been the efforts to understand their impact on health. EVALI (E-cigarette or Vaping product use Associated Lung Injury) is an emerging condition with a heterogeneous presentation with several reported cases worldwide. We mean to report a case of EVALI in a 15-year-old female Caucasian patient, who's currently attending her clinic follow-up at Bambino Gesù Pediatric Hospital in Rome. CASE PRESENTATION: The patient was admitted to the Emergency Room due to acute respiratory failure in November 2020. At admittance, she was severely dyspneic (HR 120 bpm, SatO2 75%). As she was hospitalized amid the COVID-19 pandemics, she underwent a nasopharyngeal swab for SARS-CoV2, which turned out negative, and a chest CT scan. Chest CT scan showed a central ground grass pattern with peripheral sparing. At the anamnestic recall, it was disclosed she was an e-cigarette smoker and occasional marijuana user. The microbiological work-up proved only positive for Rhinovirus. Her clinical and radiological case was discussed with our radiologist who suspected EVALI. She was assisted through HFNC, antibiotical therapy and corticosteroids with a dramatic recovery within the first 48 h. CONCLUSIONS: EVALI started being recognized a specifically nosological entity in summer 2019, with increasing cases being reported. No diagnostic criteria have been agreed upon yet, but its usual presentation includes respiratory, gastrointestinal and systemic symptoms of different degree and the diagnosis can be hypothesised in case the patient has an evocative clinical and radiological presentation and has been an E-cigarette smoker in previous 90 days. Due to the novelty of the condition and its heterogeneous presentation it is of interest to report the cases in which EVALI is identified to raise awareness about this emerging new-age disease.

Posterior fossa ependymoma in neurodevelopmental syndrome caused by a de novo germline pathogenic POLR2A variant.

Ependymoma is the third most common pediatric brain tumor. Predisposition to develop ependymomas has been reported in different hereditary diseases, but the pathogenic variants related to the familial syndromes have rarely been detected in sporadic ependymomas. De novo variants in POLR2A, the gene encoding the largest subunit of RNA polymerase II, cause a neurodevelopmental disorder with a wide range of clinical manifestations, characterized by severe infantile-onset hypotonia, developmental delay, feeding difficulties, palatal anomalies, and facial dysmorphisms. As somatic events, POLR2A mutations represent a recurrent somatic lesion in benign meningiomas. Here we describe a case of ependymoma in a 2-year-old male with a de novo pathogenic variant in POLR2A predicted to impair proper interaction of the subunit with transcription-elongation factor TFIIS, whose function is required for back-tracking of the enzyme due to elongation blocks or nucleotide misincorporation, and expected to result in an increased error and reduced elongation rates. To date, ependymoma has never been reported in patients harboring pathogenic POLR2A variants. Further information is required to explore the possibility of a differential clinical and functional impact of the pathogenic POLR2A variants and the eventual inclusion of the POLR2A neurodevelopmental disorder among the cancer predisposition syndromes with the possible development of ependymomas.

One Year of Lung Ultrasound in Children with SARS-CoV-2 Admitted to a Tertiary Referral Children's Hospital: A Retrospective Study during 2020-2021.

During the COVID-19 pandemic, the lung ultrasound (LU) turned out to be a pivotal tool to study the lung involvement in the adult population, but the same was not well evaluated in children. We detected the LU patterns through an integrated approach with clinical−laboratory features in children hospitalized for COVID-19 in relation to the temporal trend of the Italian epidemic. We conducted a retrospective study which took place at a pediatric tertiary hospital from 15 March 2020 to 15 March 2021. We compared the characteristics of the initial phase of the first COVID-19 year­in the spring and summer (15 March−30 September 2020)­and those of the second phase­in the autumn and winter (1 October 2020−15 March 2021). Twenty-eight patients were studied both in the first and in the second phase of the first COVID-19 year. The disease severity score (DSS) was significantly greater in the second phase (p = 0.015). In the second phase of the first COVID-19 year, we detected a more significant occurrence of the following LU features than in the first phase: the irregular pleural line (85.71% vs. 60.71%; p = 0.035), the B-lines (89.29% vs. 60%; p = 0.003) and the several but non-coalescent B-lines (89.29% vs. 60%; p = 0.003). The LU score correlated significantly with the DSS, with a moderate relationship (r = 0.51, p < 0.001). The combined clinical, laboratory and ultrasound approaches might be essential in the evaluation of pulmonary involvement in children affected by COVID-19 during different periods of the pandemic.

State-of-the-art review of lung imaging in cystic fibrosis with recommendations for pulmonologists and radiologists from the "iMAging managEment of cySTic fibROsis" (MAESTRO) consortium.

OBJECTIVE: Imaging represents an important noninvasive means to assess cystic fibrosis (CF) lung disease, which remains the main cause of morbidity and mortality in CF patients. While the development of new imaging techniques has revolutionised clinical practice, advances have posed diagnostic and monitoring challenges. The authors aim to summarise these challenges and make evidence-based recommendations regarding imaging assessment for both clinicians and radiologists. STUDY DESIGN: A committee of 21 experts in CF from the 10 largest specialist centres in Italy was convened, including a radiologist and a pulmonologist from each centre, with the overall aim of developing clear and actionable recommendations for lung imaging in CF. An a priori threshold of at least 80% of the votes was required for acceptance of each statement of recommendation. RESULTS: After a systematic review of the relevant literature, the committee convened to evaluate 167 articles. Following five RAND conferences, consensus statements were developed by an executive subcommittee. The entire consensus committee voted and approved 28 main statements. CONCLUSIONS: There is a need for international guidelines regarding the appropriate timing and selection of imaging modality for patients with CF lung disease; timing and selection depends upon the clinical scenario, the patient's age, lung function and type of treatment. Despite its ubiquity, the use of the chest radiograph remains controversial. Both computed tomography and magnetic resonance imaging should be routinely used to monitor CF lung disease. Future studies should focus on imaging protocol harmonisation both for computed tomography and for magnetic resonance imaging. The introduction of artificial intelligence imaging analysis may further revolutionise clinical practice by providing fast and reliable quantitative outcomes to assess disease status. To date, there is no evidence supporting the use of lung ultrasound to monitor CF lung disease.

The Role of Lung Ultrasound in Diagnosing COVID-19-Related Multisystemic Inflammatory Disease: A Preliminary Experience.

BACKGROUND: To date, there are no data regarding the systematic application of Point-of-Care Lung Ultrasound (PoC-LUS) in children with Multisystem Inflammatory Syndrome in Children (MIS-C). The main aim of this study is to show the role of Point-of-Care Lung Ultrasound as an additional aid in the diagnosis of COVID-19-related Multisystem Inflammatory Syndrome in Children (MIS-C). METHODS: Between April 2020 and April 2021, patients aged 0-18 years referred to our emergency department for fever, and later hospitalized without a specific diagnosis, underwent PoC-LUS. Ultrasound images of patients with a final diagnosis of MIS-C were retrospectively evaluated. RESULTS: Ten patients were enrolled. All were described to have pleural irregularities and B-lines. In particular: 8/10 children presented with isolated B-lines in at least half of the lung areas of interest; 8/10 presented with multiple B-lines and 3/8 had them in at least 50% of lung areas; 5/10 had a white lung appearance in at least one lung area and 1/5 had them in half of the areas of interest. Pleural effusion was described in 9/10. CONCLUSIONS: During the ongoing COVID-19 pandemic, we suggest performing PoC-LUS in febrile patients with high levels of inflammatory indices and clinical suspicion of MIS-C, or without a certain diagnosis; the finding of many B-lines and pleural effusion would support the diagnosis of a systemic inflammatory disease.