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Isolated right subsegmental peripheral bronchial atresia in a girl. Transverse chest CTs at ages 7 months (a) and 3 years (b) show progressive accumulation of secretions in the initially air-filled dilated subsegmental bronchus of the right lower lobe (arrow in a), attaining the typical features of a bronchocele (arrow in b). The child remains under clinical observation, asymptomatic at 7 years of age
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Congenital lung malformations are increasingly detected before birth. However, bronchial atresia is rarely identified in utero and not always recognized in neonates. There are two types of atresia: 1) proximal, located at the level of the mainstem or the proximal lobar bronchi, which is extremely rare and usually lethal during pregnancy, causing a...
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Citations
... Proximal bronchial atresia, which is located at the level of the mainstem or the proximal lobar bronchi, could be identified in utero US as a homogeneously hyperechogenic mass that may originate at a centrally located bronchocele. On the other hand, peripheral bronchial atresia can present as part of more complex lung malformations [33]. ...
... Bronchial and tracheal agenesis can be observed in prenatal MRI, and early diagnosis can prevent lethal outcomes once the newborn is delivered. Bronchial atresia might predispose to accumulation of fluid and tissue overexpansion, which can be demonstrated as hyperintensities on T2-W [33]. CHAOS diagnosis and fetal airway patency can be demonstrated prenatally with MRI, aiding in surgical planning [30]. ...
Congenital tracheal disorders are a heterogeneous group of pathologies characterized by noisy breathing and abnormal compressibility of the main airway. They encompass a wide variety of abnormalities and can differ in prevalence and/or incidence. Congenital tracheal abnormalities can present as impaired/abnormal cartilage development, or as extrinsic compression from other thoracic/gastrointestinal organs; they can also be classified as static or dynamic anomalies [1, 2]. While some of them may not warrant an urgent medical visit, others may acutely effect a child’s life, potentially causing airway obstruction, impairment of ventilation, respiratory distress, or cerebral hypoxia and requiring emergent management.
... Antenatal characterization of bronchial atresia is admittedly difficult because segmental and subsegmental bronchi are poorly visualized, whereas the hyperexpansion of the affected lung segment is a nonspecific finding [17]. On thin-slice T2-weighted MR sequences, demonstration of a stable tiny hyperintense round structure medially to the expanded lung segment might indicate the bronchocele and give insight to this abnormality [20]. However, the influence of fetal MRI on prenatal management of lung lesions has not proved to be overall higher than that of US, so MRI should be used selectively in these cases [19]. ...
The indications for fetal body MRI are amplifying because of the expanding possibilities of fetal and perinatal therapy. However, huge heterogeneity regarding the indications for fetal body MRI is seen among different European countries that is mostly related to local use of US, but also to local fetal MRI expertise and legislation on pregnancy termination. The purpose of this article is to summarize the precise indications for fetal MRI, excluding the central nervous system. MRI indications arise from the sonographic findings, based on the operator’s experience and the various practices in the countries and institutions represented on the European Society of Paediatric Radiology Fetal Task Force. We also highlight the strengths and weaknesses of fetal US and MRI of the fetal body.
... Bronchial atresia is frequently asymptomatic at birth and in early childhood. It is often discovered incidentally in adolescence or adulthood on chest imaging obtained for other indications [37,39]. In contradistinction to congenital lobar overinflation, no ball-valve mechanism is present in bronchial atresia because the airway is completely occluded and not just narrowed [14]. ...
... The dilated bronchus can be completely opacified with fluid or mucus or contain an air-fluid level [37,40]. Bronchial atresia complicated by recurrent infection is treated with lobectomy or segmentectomy [37,39]. Some propose observation rather than surgery for asymptomatic bronchial atresia [41]. ...
Congenital lung lesions are a rare group of developmental pulmonary abnormalities that are often first identified prenatally on routine second-trimester US. Congenital pulmonary airway malformation (CPAM) is the most common anomaly while others include bronchopulmonary sequestration, congenital lobar overinflation, bronchogenic cyst and bronchial atresia. Clinical presentation is highly variable, ranging from apparent in utero resolution to severe mass effect with resultant hydrops fetalis and fetal demise. Differentiation among these lesions can be challenging because overlapping imaging features are often present. The roles of the radiologist are to identify key imaging findings that help in diagnosing congenital lung lesions and to recognize any ominous features that might require prenatal or perinatal intervention. High-resolution US and complementary rapid-acquisition fetal MRI provide valuable information necessary for lesion characterization. Postnatal US and CT angiography are helpful for lesion evaluation and for possible surgical planning. This article reviews the embryology of the lungs, the normal prenatal imaging appearance of the thorax and its contents, and the prenatal and neonatal imaging characteristics, prognosis and management of various congenital lung lesions.
... Bronchial atresia (BA) is a rare congenital pulmonary abnormality consisting of focal obliteration of a bronchus with normal architecture of the distal lung and hyperinflation of the involved segment. Two types of bronchial atresia are described: (1) proximal, located at the level of the mainstem or the proximal lobar bronchi which is usually associated with fetal demise or early postnatal mortality, and (2) peripheral, involving the segmental or subsegmental bronchi [1]. Peripheral bronchial atresia may be isolated or part of a complex congenital lung malformation, in particular a microcystic congenial pulmonary airway malformation (CPAM) [1]. ...
... Two types of bronchial atresia are described: (1) proximal, located at the level of the mainstem or the proximal lobar bronchi which is usually associated with fetal demise or early postnatal mortality, and (2) peripheral, involving the segmental or subsegmental bronchi [1]. Peripheral bronchial atresia may be isolated or part of a complex congenital lung malformation, in particular a microcystic congenial pulmonary airway malformation (CPAM) [1]. Peripheral bronchial atresia is frequently diagnosed on postnatal computed tomography (CT) examinations after prenatal imaging reveals a congenital lung lesion [1]. ...
... Peripheral bronchial atresia may be isolated or part of a complex congenital lung malformation, in particular a microcystic congenial pulmonary airway malformation (CPAM) [1]. Peripheral bronchial atresia is frequently diagnosed on postnatal computed tomography (CT) examinations after prenatal imaging reveals a congenital lung lesion [1]. ...
Purpose
Peripheral bronchial atresia is a pulmonary abnormality diagnosed on postnatal computed tomography after prenatal imaging reveals a congenital lung lesion. Debate regarding management of this abnormality prompted us to review our institution’s practice patterns and outcomes.
Methods
All patients diagnosed with bronchial atresia were assessed from 6/2014 to 7/2020. Pediatric radiologists were surveyed to delineate computed tomography criteria used to diagnose peripheral bronchial atresia. Criteria were applied in an independent blinded review of postnatal imaging. Data for patients determined to have peripheral bronchial atresia and at least an initial pediatric surgical evaluation were analyzed.
Results
Twenty-eight patients with bronchial atresia received at least an initial pediatric surgical evaluation. Expectant management was planned for 22/28 (79%) patients. Two patients transitioned from an expectant management strategy to an operative strategy for recurrent respiratory infections; final pathology revealed bronchial atresia in both. Six patients were initially managed operatively; final pathology revealed bronchial atresia (n = 3) or congenital lobar overinflation (n = 3).
Conclusions
Peripheral bronchial atresia can be safely managed expectantly. A change in symptoms is suspicious for alternate lung pathology, warranting further workup and consideration for resection.
Level of evidence
Level IV.
... Tipik olarak üst lobların apikal veya apikoposterior segmentinde yer alır. Rekürren enfeksiyonu olan hastalarda cerrahi tedavi uygulanabilir [14]. ...
... Isolated bronchial atresia most commonly affects segmental and subsegmental bronchi. Central or lobar bronchial atresia is rare and usually lethal (hyperinflation of involved lung precludes adequate normal contralateral lung development), and hence, is not imaged [13]. As stated, microscopic examination has revealed that bronchial atresia coexists with nearly all cases of BPS and most cases of CPAM [2]. ...
... Preoperative airway evaluation is therefore essential for accurate patient counseling and surgical decision-making [18]. Overall, morbidity and mortality is increased in type II PA sling relative to type I [13,15]. ...
... While the presence of cysts in a congenital lung lesion suggests CPAM, cysts are also seen in other lesions. For example, a central mucocele/bronchocele, an imaging hallmark of bronchial atresia, can be mistaken for a cyst in a CPAM [13]. Given imaging overlap with other entities, postnatal imaging evaluation with CT is necessary for more definitive characterization when CPAM is suspected. ...
Congenital lung malformations represent a spectrum of abnormalities that can overlap in imaging appearance and frequently coexist in the same child. Imaging diagnosis in the neonatal period can be challenging; however, the recognition of several archetypal radiographic patterns can aid in narrowing the differential diagnosis. Major radiographic archetypes include (1) hyperlucent lung, (2) pulmonary cysts, (3) focal opacity and (4) normal radiograph. Here we review the multimodality imaging appearances of the most commonly seen congenital lung malformations, categorized by their primary imaging archetypes. Along with the congenital lung malformations, we present several important imaging mimickers.
... The presence of a cystic structure thought to represent a mucous plug adjacent to proximal bronchial atresia (BA) has been described as a mucocele or bronchocele in a small number of BA and congenital high airway obstruction sequence cases (5,7,11,12,15,19,20). However, the hyperintense bronchus sign (HBS) refers to a distension of the segmental and subsegmental bronchi and, to our knowledge, has not received any specific attention so far. ...
Background Fetal MRI-based differential diagnosis of congenital lung malformations is difficult because of the paucity of well-described imaging markers. Purpose To characterize the hyperintense bronchus sign (HBS) in in vivo fetal MRI of congenital lung malformation cases. Materials and Methods In this retrospective two-center study, fetal MRI scans obtained in fetuses with congenital lung malformations at US (January 2002 to September 2018) were reviewed for the HBS, a tubular or branching hyperintense structure within a lung lesion on T2-weighted images. The frequency of the HBS and respective gestational ages in weeks and days were analyzed. Areas under the curve (AUCs), 95% CIs, and P values of the HBS regarding airway obstruction, as found in histopathologic and postnatal CT findings as the reference standards, were calculated for different gestational ages. Results A total of 177 fetuses with congenital lung malformations (95 male fetuses) and 248 fetal MRI scans obtained at a median gestational age of 25.6 weeks (interquartile range, 8.9 weeks) were included. The HBS was found in 79% (53 of 67) of fetuses with bronchial atresia, 71% (39 of 55) with bronchopulmonary sequestration (BPS), 43% (three of seven) with hybrid lesion, 15% (six of 40) with congenital cystic adenomatoid malformation, and 13% (one of eight) with bronchogenic cyst at a median gestational age of 24.9 weeks (interquartile range, 9.7 weeks). HBS on MRI scans at any gestational age had an AUC of 0.76 (95% CI: 0.70, 0.83; P = .04) for the presence of isolated or BPS-associated airway obstruction at histopathologic analysis and postnatal CT. The AUC of HBS on fetal MRI scans obtained until gestational age of 26 weeks (AUC, 0.83; 95% CI: 0.75, 0.91; P < .001) was significantly higher (P = .045) than that for fetal MRI scans obtained after gestational age 26 weeks (AUC, 0.69; 95% CI: 0.57, 0.80; P = .004). Conclusion The hyperintense bronchus sign is a frequently detectable feature at fetal MRI and is associated with airway obstruction particularly before gestational age 26 weeks. © RSNA, 2021 Online supplemental material is available for this article. See also the editorial by Dubinsky in this issue.
... CT scan is considered the gold standard modality. 2,3,[5][6][7]9,10 Extrinsic lesions Non-vascular lesions Foregut duplication cysts. They arise during pulmonary development due to abnormal budding of the embryonic foregut and the tracheobronchial tree. ...
Large airway diseases" is being used as an all-encompassing phrase to describe a broad spectrum of pathological entities, which involves the trachea, main, lobar, and segmental bronchi of up to 3 mm diameter. Imaging modalities such as radiography, computed tomography, and magnetic resonance imaging contribute to the identification and diagnosis of each entity. Knowledge of clinical information, normal cross-sectional anatomy, and imaging characteristics of large airway diseases is necessary for appropriate radiologic evaluation. This review provides information about congenital and acquired diseases of the large airways in the pediatric population.
... This uncommon congenital lesion occurs when there is interruption of the lumen of a lobar or segmental bronchus, usually associated with the development of a mucocele, as well as decreased attenuation (and often hyperexpansion) of the affected part of the lung (25). CT and MRI show a branching tubular structure, usually arising at or close to the hilum (Figure 5). ...
Various imaging techniques may be used to diagnose airway obstruction in children. Digital radiography, computed tomography and magnetic resonance imaging are the most important modalities, but the choice of technique will depend on the level and nature of suspected obstruction, as well as patient-specific factors such as age and ability to cooperate. This review examines the forms of airway obstruction that are commonly encountered in childhood.
... With the advancement of science and technology, the development and application of MSCT has achieved higher spatial resolution than with traditional CT, and the presentation of some small blood vessels has become even more pronounced. Combined with CT volumetric technology and data acquisition technology, the bronchus and the entire lungs of children can be observed in multiple orientations and angles, reducing the artifacts that are caused by breathing and allowing the best imaging results to be obtained [27]. To date, no relevant local or international study has provided evidence for the use of MSCT for diagnosing bronchial dysplasia in premature infants. ...
Background
The aim of this study was to investigate the diagnostic value of multi-slice spiral computed tomography (MSCT) for bronchial dysplasia in premature infants.
Material/Methods
A retrospective analysis of 248 premature infants who were highly suspected to have bronchial dysplasia and were admitted to our hospital from 2015 onwards was conducted. We observed bronchus morphologies, sizes, and tissue characteristics using fiberoptic bronchoscopy (FB) as the criterion standard for diagnosis. We calculated the sensitivity, specificity, and diagnostic compliance of MSCT in the diagnosis of bronchial dysplasia.
Results
Thoracic computed tomography mainly revealed capsular bubbles. The translucency of the 2 lungs was reduced, and extensive and local ground-glass changes were observed. Imaging findings mostly included strip or honeycomb-like shadows. Pleural thickening and pleural effusion were rare. MSCT was able to establish a diagnosis in 92 cases (37.10%) of bronchopulmonary cysts, 69 cases (27.82%) of congenital pulmonary emphysema, 31 cases (12.50%) of bronchial atresia, 1 case (0.40%) of congenital cystadenoma malformation, and 3 cases (1.21%) of giant tracheal bronchitis. Another 52 children (20.97%) were found to have conventional pulmonary inflammation. The sensitivity of MSCT in the diagnosis of bronchial dysplasia was 88.21%, the specificity was 75.00%, and the diagnostic compliance was 86.29%. There was a significant difference between the MSCT and FB findings in the diagnosis of bronchial hypoplasia (P<0.001).
Conclusions
MSCT has great utility in the diagnosis of bronchial dysplasia in premature infants and may become an excellent method for diagnosing bronchial dysplasia in the future.
