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Isolated overinflation (bronchial atresia) in an 11-month-old boy. a Axial T2-weighted image shows a hypointense lung area (arrowheads) with rarefication of the pulmonary markings, central fluid-filled round and linear bronchial structures (*, mucoceles), and some consolidation (arrow). b Coronal contrast-enhanced image at pulmonary peak enhancement shows the pulmonary lesion as a perfusion defect
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BackgroundA radiation-free advanced imaging modality is desirable for investigating congenital thoracic malformations in young children.Objective
To describe magnetic resonance imaging (MRI) findings of congenital bronchopulmonary foregut malformations and investigate the ability of lung MRI for their classification.Materials and methodsThis is a r...
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Citations
... The current gold standard for postnatal CLA imaging is chest computed tomography (CT), but this has disadvantages, such as exposure to ionizing radiation and need for a contrast agent to visualise vascularisation of the lesion [11][12][13]. Magnetic resonance imaging (MRI) could be a safe alternative, being free of ionizing radiation and comparable at showing lung structure, as recently seen in a postnatal cohort of CLA patients [14,15]. To date, no studies on the long-term follow-up of CLA using MRI have been reported. ...
... MRI was found to be comparable to CT for the visualisation of all CLA-related lung structures, except for vascularisation [14]. A study by Kellenberger et al compared postnatal contrast-enhanced CT to contrast-enhanced MRI, and although this study describes comparable findings on the two modalities, contrast enhancement is described as indispensable [15]. In contrast, we found that MRI was able to identify CLA-related vascular abnormalities in all patients. ...
Objectives:
Follow-up of congenital lung abnormalities (CLA) is currently done with chest computer tomography (CT). Major disadvantages of CT are exposure to ionizing radiation and need for contrast enhancement to visualise vascularisation. Chest magnetic resonance imaging (MRI) could be a safe alternative to image CLA without using contrast agents. The objective of this cohort study was to develop a non-contrast MRI protocol for the follow-up of paediatric CLA patients, and to compare findings on MRI to postnatal CT in school age CLA patients.
Methods:
Twenty-one CLA patients, 4 after surgical resection and 17 unoperated (mean age 12.8 (range 9.4-15.9) years), underwent spirometry and chest MRI. MRI was compared to postnatal CT on appearance and size of the lesion, and lesion associated abnormalities, such as hyperinflation and atelectasis.
Results:
By comparing school-age chest MRI to postnatal CT, radiological appearance and diagnostic interpretation of the type of lesion changed in 7 (41%) of the 17 unoperated patients. In unoperated patients, the relative size of the lesion in relation to the total lung volume remained stable (0.9% (range - 6.2 to + 6.7%), p = 0.3) and the relative size of lesion-associated parenchymal abnormalities decreased (- 2.2% (range - 0.8 to + 2.8%), p = 0.005).
Conclusion:
Non-contrast-enhanced chest MRI was able to identify all CLA-related lung abnormalities. Changes in radiological appearance between MRI and CT were related to CLA changes, patients' growth, and differences between imaging modalities. Further validation is needed for MRI to be introduced as a safe imaging method for the follow-up of paediatric CLA patients.
Key points:
• Non-contrast-enhanced chest MRI is able to identify anatomical lung changes related to congenital lung abnormalities, including vascularisation. • At long-term follow-up, the average size of congenital lung abnormalities in relation to normal lung volume remains stable. • At long-term follow-up, the average size of congenital lung abnormalities associated parenchymal abnormalities such as atelectasis in relation to normal lung volume decreases.
... For several years, many tertiary paediatric hospitals have routinely relied on MRI as the second-line imaging modality in babies with congenital heart disease when echocardiography does not provide all necessary information for diagnosis and planning treatment [65][66][67]. Recent developments of MR technology have improved the image quality and allow for better evaluation of cardiovascular anatomy, blood flow, airways and lungs, which makes comprehensive advanced imaging of the neonatal chest possible [68,69]. In the following section, we describe our MRI approach to complex congenital heart disease and congenital lung malformations. ...
... In symptomatic babies, immediate further imaging may be necessary for complete assessment and planning surgery or other intervention. The parenchymal characteristics of congenital lung malformations are well delineated as consolidation, air-filled macrocysts and hyperinflated lung areas on respiratorygated conventional fast spin echo or ultra-short echo time sequences (Figs. 14 and 15) [68,73]. In the first days of life, cysts and "hyperinflated" lesions may still contain fluid. ...
... Fluid-filled lesions and mucoid impaction resulting from airway obstruction are more conspicuous on T2-weighted fast spin echo compared to proton density weighted ultrashort echo-time images (Fig. 15). Dynamic contrastenhanced lung imaging may help localise and characterise lung lesions with systemic blood supply (e.g., bronchopulmonary sequestration, isolated overinflation) and help differentiate them from atelectasis due to sedation [68]. Most congenital lung lesions present as perfusion defects during peak lung enhancement (Fig. 15) and bronchopulmonary sequestrations show delayed enhancement during the systemic arterial phase [68]. ...
Advanced cardiorespiratory imaging of the chest with ultrasound (US), computed tomography (CT) and magnetic resonance imaging (MRI) plays an important role in diagnosing respiratory and cardiac conditions in neonates when radiography and echocardiography alone are not sufficient. This pictorial essay highlights the particularities, clinical indications and technical aspects of applying chest US, cardiac CT and cardiorespiratory MRI techniques specifically to neonates, summarising the first session of the European Society of Paediatric Radiology’s cardiothoracic task force.
... 1,2 Estimated annual incidence of congenital lung malformations (CLM) is 30-42 per 100,000 live births and of congenital diaphragmatic abnormalities of which most common is congenital diaphragmatic hernia (CDH) is 1 in 3000 live births. 1,3 CLM include variety of developmental problems of pulmonary system and include congenital pulmonary airway malformation (CPAM), bronchial cysts, congenital segmental or lobar emphysema, congenital cystic adenomatoid malformation (CCAM), bronchopulmonary sequestration (BPS) and vascular malformations. 4 CDH is the birth defect in diaphragm with herniation of intraabdominal viscera in chest cavity causing pulmonary hypoplasia and pulmonary hypertension which are main determines of morbidity and mortality and in some cases, it is also associated with other anomalies of genitourinary, cardiovascular and brain. 5,6 It has three types, posterolateral Boc dalek hernia, anterior Morgagni hernia and hiatal hernia. ...
Objective: To describe the clinical spectrum of presentation and outcome of children with congenital thoracic malformations beyond neonatal age. Methodology: Cross sectional study conducted at inpatient department of National institute of child health from Jan-Dec 2021. All patients hospitalized in study duration with diagnosis of congenital thoracic formations from 1 month till 12 years of age were enrolled. History, examination, laboratory tests, treatment and outcome were recorded. Results: Total 44 children were enrolled with mean age of 7.7+10.8 months. Common congenital thoracic malformations were congenital diaphragmatic hernia 24(54.5%), congenital cystic adenomatoid malformation 6(13.6%), congenital lobar emphysema5(11.4%) and eventration of diaphragm 4(9.1%). Three (6.9%) patients had associated cardiac anomalies. Surgical intervention was done in 33 (75%) patients with a postoperative survival rate of 95.4%. Conclusion: Congenital diaphragmatic hernia and cystic adenomatoid malformation are common congenital thoracic malformations that present beyond neonatal age and are associated with good postsurgical outcome. Keywords: congenital diaphragmatic hernia, congenital cystic adenomatoid malformation, beyond neonate
... In particular, the characteristic bronchocele is typically well-visualized on T2-weighted MR sequences as a tubular T2-hyperintense structure, with relative hypointense signal in the surrounding pulmonary segment [7] (Fig. 7). Bronchial atresia can occur on a spectrum with other congenital lung lesions including bronchopulmonary sequestration; therefore, CT and MRI might be performed with contrast agent, and CT angiography and MR angiography protocols are sometimes combined with airway protocols to evaluate the underlying vascular anatomy [29]. ...
Infants and children often present with respiratory symptoms referable to the airway. For these pediatric patients, airway imaging is frequently performed to evaluate for underlying disorders of the large airway. Various imaging modalities have been used to evaluate the pediatric large airway, and pediatric airway imaging techniques have continued to evolve. Therefore, clear understanding of the status and new advances in pediatric large airway imaging is essential for practicing radiologists to make timely and accurate diagnoses, which can lead to optimal pediatric patient management.
... Several characteristic imaging features of bronchopulmonary malformations are evident on both pre-and postnatal imaging [6,7,9,[15][16][17]. ...
... Sequences employed include: two-dimensional (2-D) steadystate free precession; single-shot fast spin echo; respiratorygated proton-density and T1-and T2-weighted fast spin echo; and fast spoiled gradient echo acquisition or 3-D volume T1weighted inversion pulse sequence with fat suppression, which can be obtained both pre-and post-contrast and reconstructed into fat, water and in-and out-of-phase images. Dynamic multiphasic angiographic sequences include 3-D gradient echo fatsuppressed respiratory/cardiac-gated sequences or time-resolved MR angiography [12,16]. A more recent addition has been the development of ultrashort echo-time T1-weighted sequences (both pre-and post-contrast), which greatly improve the visibility of lung parenchyma. ...
... Magnetic resonance chest imaging studies typically take approximately 45 min to 1 h to complete [16]. Children undergoing these MR examinations usually require sedation or anesthesia. ...
Congenital lung malformations are most often identified on prenatal US screening. Fetal MRI is often performed to further evaluate these lesions. Although some of these lesions might cause prenatal or early postnatal symptoms that require urgent management, the majority are asymptomatic at birth and might be subtle or invisible on chest radiographs. Postnatal imaging is frequently deferred until 3–6 months of age, when surgery or long-term conservative management is contemplated. High-quality imaging and interpretation is needed to assist with appropriate decision-making. Contrast-enhanced chest CT, typically with angiographic technique, has been the usual postnatal imaging choice. In this review, the author discusses and illustrates the indications and use of postnatal MR imaging for bronchopulmonary malformations as well as some differential diagnoses and the advantages and disadvantages of MR versus CT.
... Perfusion imaging with a dynamic 3D gradient echo sequence (TRICKS) can be added to the MRI protocol. 13,14 Although these sequences work well in pulmonary diseases with increased signal in the lung tissue or in fluid collections, they are not sufficient in the opposite case. 7 The extremely short T2* of the lung demands a fast signal acquisition following excitation before the signal decays into the noise level. ...
... 32 Prenatally detected lesions should be confirmed postnatally by CT or MRI, for characterisation of the lesion, planning surgery or as base line for follow-up of asymptomatic lesions. 13,34 The UTE sequence allows for the detection of parenchymal lung abnormalities including abnormalities of aeration and perfusion in segmental or lobar hyperinflation and better delineation of cystic change in cases with CPAM or hybrid lesions compared to the other lung MRI sequences (Figs 11e14). ...
Lung magnetic resonance imaging (MRI) is considered to be challenging, because the low proton density of the tissue, fast signal decay, and respiratory artefacts hamper adequate image quality. MRI of the lungs and thorax is increasingly used in the paediatric population, because it is a radiation-free alternative to chest CT. Recently, ultrashort echo-time (UTE) sequences have been introduced into clinical MRI protocols, in order to improve the contrast-to-noise ratio due to reduced susceptibility artefacts and to depict structural alterations comparable to CT. The purpose of this review is to provide an overview of various clinical conditions and pathologies in the paediatric chest depicted by an UTE sequence, the so-called three-dimensional (3D) Cones sequence, in comparison with conventional MRI sequences. Besides describing typical features of cystic fibrosis, we present UTE application in other more or less common paediatric lung pathologies, for instance, interstitial pneumopathies, pulmonary infections, and congenital pulmonary malformations.
... due to trauma [75] or torsion of vascular pedicle in ELS [76]. A recent article by Kellenberger et al. illustrated a dedicated lung MRI protocol for a comprehensive post-natal structural and perfusion assessment of CBPFMs (including PS), showing a perfusion defect of all lesions at peak pulmonary enhancement and a delayed peak of noncystic lesions compared to normal lung tissue [77]. ...
Pulmonary sequestration consists of a nonfunctioning mass of lung tissue, either sharing the pleural envelope of the normal lung (intralobar) or with its own pleura (extralobar), lacking normal communication with the tracheobronchial tree and receiving its arterial supply by one or more systemic vessels. It is the second most common congenital lung anomaly according to pediatric case series, but its real prevalence is likely to be underestimated, and imaging plays a key role in the diagnosis and treatment management of the condition and its potential complications. We will give a brief overview of the pathophysiology, clinical presentation and imaging findings of intra- and extralobar pulmonary sequestration, with particular reference to multidetector computed tomography as part of a powerful and streamlined diagnostic approach.
*****TEMPORARY LINK TO FULL-TEXT ARTICLE (VALID BEFORE JANUARY 29, 2021): https://authors.elsevier.com/a/1cDJB3CaTZJiBr
Congenital lung malformations (CLMs) are rare developmental anomalies of the lung, including congenital pulmonary airway malformations (CPAM), bronchopulmonary sequestration, congenital lobar overinflation, bronchogenic cyst and isolated congenital bronchial atresia. CLMs occur in 4 out of 10,000 live births. Postnatal presentation ranges from an asymptomatic infant to respiratory failure. CLMs are typically diagnosed with antenatal ultrasonography and confirmed by chest CT angiography in the first few months of life. Although surgical treatment is the gold standard for symptomatic CLMs, a consensus on asymptomatic cases has not been reached. Resection, either thoracoscopically or through thoracotomy, minimizes the risk of local morbidity, including recurrent infections and pneumothorax, and avoids the risk of malignancies that have been associated with CPAM, bronchopulmonary sequestration and bronchogenic cyst. However, some surgeons suggest expectant management as the incidence of adverse outcomes, including malignancy, remains unknown. In either case, a planned follow-up and a proper transition to adult care are needed. The biological mechanisms through which some CLMs may trigger malignant transformation are under investigation. KRAS has already been confirmed to be somatically mutated in CPAM and other genetic susceptibilities linked to tumour development have been explored. By summarizing current progress in CLM diagnosis, management and molecular understanding we hope to highlight open questions that require urgent attention.
Introduction:
Although historically challenging to perform in the lung, technological advancements have made Magnetic Resonance Imaging (MRI) increasingly applicable for pediatric pulmonary imaging. Furthermore, a wide array of functional imaging techniques has become available, to be leveraged alongside structural imaging for increasingly sensitive biomarkers, or outcome measures in the evaluation of novel therapies.
Areas covered:
In this review, recent technical advancements and modern methodologies for structural and functional lung MRI are described. These include ultrashort echo time (UTE) MRI, free-breathing contrast agent-free, functional lung MRI, and hyperpolarized gas MRI, amongst other techniques. Specific examples of the application of these methods in children are provided, principally drawn from recent research in asthma, bronchopulmonary dysplasia, and cystic fibrosis.
Expert opinion:
Pediatric lung MRI is rapidly growing, and is well poised for clinical utilization, as well as continued research into early disease detection, disease processes, and novel treatments. Structure/function complementarity make MRI especially attractive as a tool for increased adoption in the evaluation of pediatric lung disease. Looking toward the future, novel technologies, such as low-field MRI and artificial intelligence, mitigate some of the traditional drawbacks of lung MRI and will aid in improving access to MRI in general, potentially spurring increased adoption and demand for pulmonary MRI in children.
