Selection of the most appropriate CT protocol for each individual case is paramount in imaging of the airways in children. Ultimately, the best protocol is the one that provides the most relevant information at the lowest radiation burden possible. The indications include: (1) congenital bronchial anomalies (e.g. accessory bronchi, bronchial hypoplasia and atresia, and bronchopulmonary foregut malformations), (2) tracheomalacia, (3) tracheobronchial strictures (congenital and acquired) or tumours, and (4) peripheral (small) airways disease.
Tracheobronchial anomalies
Approximately 1–12% of adult patients who undergo bronchoscopy demonstrate some form of congenital variant [1, 11]. Tracheobronchial anomalies may be associated with recurrent episodes of pulmonary infection and airway obstruction and usually go undiagnosed with conventional imaging [1, 12].
Tracheal bronchus
Tracheal bronchus, with an incidence of 0.1–5%, represents an aberrant bronchus that usually arises from the right tracheal wall above the carina and ventilates the apical segment of the upper lobe (Fig. 13) [1, 13]. If the right upper-lobe bronchus has a normal trifurcation then the tracheal bronchus is supernumerary and may end blindly; it is also called a tracheal diverticulum. Left tracheal bronchus has also been reported [13]. Its presence may be associated in some patients with other abnormalities of the lung or within the spectrum of the VACTREL (V vertebral, A anorectal atresia, C cardiac, T tracheobronchial, R renal, E esophageal, L lumbosacral/limb abnormalities) association. Diagnosis of tracheal bronchus should be considered early in the clinical course of intubated patients with recurrent right upper-lobe complications [11]. Coronal MPRs are particularly helpful in demonstrating the presence of a tracheal bronchus, which may go undetected on axial images. Findings can be additionally supported with VB.
Bronchial atresia
Bronchial atresia may present with a variety of findings depending on the child’s age (water-density mass in newborns, bronchocele and air-trapping during childhood, and solitary pulmonary nodule or area of emphysema in adults). In doubtful cases, MPRs help identify mucoid impaction and bronchocele as a branching structure radiating from the hilum [13].
Bronchopulmonary foregut malformations
These are anomalies of pulmonary development that are due to abnormal budding of the embryonic foregut and tracheobronchial tree. They include duplication cysts characterized by an isolated portion of lung tissue communicating with the upper gastrointestinal tract or the central nervous system such as bronchogenic cysts, enteric cysts, and neurenteric cysts. Symptoms are usually provoked by the size and location of the cysts, which may cause compression of the trachea or bronchi leading to distal collapse and air trapping. Infection is less commonly encountered.
Pulmonary underdevelopment
The spectrum of pulmonary underdevelopment includes agenesis of the bronchus and lung (pulmonary agenesis), presence of a rudimentary blind-ending bronchus without lung tissue (pulmonary aplasia) and bronchial hypoplasia with reduction of lung tissue of variable degree (pulmonary hypoplasia) (Fig. 1) [13].
Scimitar syndrome
The scimitar syndrome, also called venolobar syndrome and hypogenetic lung syndrome, is a rare congenital cardiovascular anomaly involving the right lung. It consists of ipsilateral anomalous pulmonary drainage of part of the right lung into the inferior vena cava, hypoplasia of the right lung, dextrorotation of the heart, hypoplasia of the right pulmonary artery, and anomalous systemic arterial supply to the lower lobe of the right lung from the subdiaphragmatic aorta or its main branches. It may be associated with abnormal bronchial anatomy, abnormal diaphragm, hemivertebrae, and anomalies of the genitourinary tract. CT with MPRs shows the size of the right hemithorax, the associated bronchial anomalies, and the anomalous pulmonary (scimitar) vein [13].
Sequestration spectrum
The sequestration spectrum includes congenital pulmonary airway malformations (CPAMs), anomalies previously termed congenital cystic adenomatoid malformations, the pulmonary sequestrations and also hybrid lesions that histologically consist of both entities (Figs. 2 and 3). CT defines their location, extent and anatomical relationships, and evaluates their density. MPRs and VRs can impressively demonstrate the arterial feeder from a subphrenic aortic branch and detect the venous drainage into the systemic or pulmonary circulation for the extra- and intralobar sequestrations, respectively (Fig. 3). Enhancement of the cystic wall implies infection.
Tracheomalacia
Tracheomalacia is a condition that refers to softening of the tracheal wall. This is caused from an abnormality of the cartilaginous ring and hypotonia of the myoelastic elements. There is dynamic collapse of the trachea during expiration which leads to airway obstruction with development of wheeze, cough, stridor, dyspnoea, cyanosis and recurrent respiratory infections. Primary tracheomalacia is thought to be caused by congenital immaturity of the tracheal cartilage and may be associated with other developmental defects, such as a vascular ring/compression or tracheo-oesophageal fistula. The natural history shows that it improves by the end of the first year. In secondary tracheomalacia, previously normal cartilage undergoes degeneration [13].
Tracheobronchial strictures
Congenital tracheal stenosis
Congenital tracheal stenosis is a rare disorder characterized by a fixed tracheal narrowing caused by complete tracheal cartilage rings (Fig. 4). Approximately 50% of congenital tracheal stenoses are focal, located usually in the lower third of the trachea, 30% are generalized and 20% are funnel-shaped [13]. The bronchi are usually of normal size. This abnormality which may be associated with other anomalies, more commonly pulmonary artery sling, should be suspected when a distal tracheal stenosis is recognized in an infant [13].
Congenital lobar emphysema/overinflation
Areas of malacia or stenosis of the bronchial cartilage are thought to be the aetiological factors in the development of congenital lobar emphysema/overinflation through a check-valve mechanism, which is characterized by progressive distension/hyperinflation of a lobe, most commonly the left upper lobe. CT will typically show hyperinflation of the affected lobe without destruction of the alveolar walls and will help to differentiate this condition from others (Fig. 14).
Acquired tracheobronchial stenoses
Acquired tracheobronchial stenoses may result from prolonged intubation (post-intubation webs) or at the anastomosis sites following surgical intervention/lung transplantation. In patients in whom the axis of the stenosis is vertical or slightly oblique it is usually difficult to perceive on the axial images alone, and MPRs/VRs are useful adjunctive tools (Fig. 5).
Compression of the airways of cardiovascular origin
This is a relatively common complication of cardiovascular diseases that may go unrecognized for some time. Compression is caused either because of an underlying anomalous anatomical relationship between the tracheobronchial tree and the vascular structures, e.g. a double aortic arch, or is extrinsic, caused by enlarged cardiac or pulmonary vascular structures, e.g. congenital heart disease, dilated pulmonary arteries (Fig. 6). In children with unexplained respiratory distress, stridor, wheezing, dysphagia and apnoea, a high index of suspicion is required to identify these lesions that cause mechanical airway compression [14, 15].
A vascular ring is an aortic arch anomaly in which the trachea and the oesophagus are completely surrounded by vascular structures. They are formed because components of the aortic arch complex persist or regress abnormally. If the airway is incompletely encircled then a vascular sling is produced [13–15].
The most common and clinically serious type of vascular ring is the double aortic arch, which represents a persistence of both right and left embryonic fourth arches joining the aortic portion of the truncoaortic sac to their respective dorsal aortae [14]. It typically consists of a right and a left arch that encircle the trachea and oesophagus in a tight ring, joining distally to form a common descending aorta (Fig. 11). Variations of this anomaly have been reported, e.g. hypoplasia of one arch [13].
Another vascular sling can be produced by an abnormal origin of the left pulmonary artery from the right pulmonary artery (Fig. 12). As the former crosses to the left, it encircles and compresses the right main bronchus and the trachea and occasionally the left main bronchus. This condition is usually associated with other congenital anomalies [14].
Foreign body aspiration
This is one of the most common and potentially life threatening events in children, accounting for 7% of lethal accidents in infants aged 1–3 years [16]. MDCT of the chest for the detection of radiolucent foreign bodies is not the investigation of first choice and not routinely used unless a pulmonary infiltrate that fails to resolve within the normal recovery period of 10–14 days is present [16]. It is vital to remember that actual bronchoscopy should be performed even in cases of low clinical suspicion and no direct history since the risk of overlooking foreign body aspiration may be lethal and may cause long-term pulmonary damage.
Local extension of neoplasms
MPRs/VRs with coevaluation of the axial images are the method of choice for staging of hilar or mediastinal neoplasms. They demonstrate interfaces accurately, e.g. the relationship of the lesions to the bronchovascular walls and the presence of mediastinal lymph nodes, since they maintain voxel density values (Figs. 7 and 8). More complex rendering techniques are not usually required [3].
Peribronchial air collections
MPRs/VRs are helpful in demonstrating small extraluminal air collections and leakages of surgical anastomoses, especially in orthotopic lung or heart/lung transplant patients (Fig. 9). They can impressively show fistulous paths that may be indiscernible on axial images and affect patient management [3]. Additionally, by correlating with VB the precise location of the leak can be traced prior to actual bronchoscopy.
Peripheral airways
MPRs/VRs using MDCT technology have achieved more accurate visualization of the distal airways. The VR technique is applied in thin, overlapping, transverse reconstructed sections using a high-resolution algorithm. This is particularly helpful in the search for bronchiectasis where a single oblique MPR/VR section may include the whole portion of the affected bronchi (Fig. 10). Additionally, when planning the best approach for tissue sampling the relation between peripheral nodules and the afferent bronchus is better appreciated using the MIP/VR sections. Although the applications of conventional HRCT for the evaluation of the peripheral airways are beyond the scope of this review article, the value of HRCT should not be overlooked. When performed individually, HRCT is considerably less of a radiation burden than MDCT (Table 6), but with the standard HRCT protocol, limited slices are performed and the imaging of the mediastinum is insufficient. On the contrary, the Combiscan MDCT protocol with the possibility of HR reconstructions allows detailed parenchymal evaluation of the whole of the chest and simultaneous imaging of the mediastinum; however, there is the disadvantage of extra radiation burden (Table 6).