Congenital airway anomalies are relatively common and present in 1–12% of the general population at bronchoscopy.Reference Ghaye, Szapiro, Fanchamps and Dondelinger1 The increasing prevalence is thought to be due to increasing frequency and improved quality of medical imaging.Reference Ghaye, Szapiro, Fanchamps and Dondelinger1 Depending on the type of anomaly and degree of airway involvement, these anomalies may present in infancy or remain undiagnosed until adulthood.Reference Chaddha, Chang and Lee2
Knowledge of expected normal anatomy and the major anomalous patterns is necessary to establish the correct diagnosis and appropriately guide treatment in symptomatic patients.
In this brief report, we present a case of type 2 bridging bronchus with left pulmonary artery sling in an adult patient with recurrent symptoms. A discussion of the published findings of bridging bronchial anomalies will follow.
Case report
A 20-year-old male patient was referred to our institution for evaluation of tracheal stenosis. This was initially diagnosed in infancy when the patient presented with failure to thrive and poor oral intake. At the time of his presentation to our institution, the patient reported intermittent dyspnea which was often exacerbated by humidity during the summer months. He had undergone balloon dilatation of the distal trachea a year prior. At the time of the current presentation of dyspnea, he underwent clinical assessment, bronchoscopy, and CT examination.
A remote chest radiograph shows asymmetric hypoplasia of the right lung with subtle, smooth, and long segment stenosis of the airway. The pseudo-carina appeared splayed at the T6–T7 thoracic vertebral level.
CT demonstrated abnormalities of the airway and pulmonary arteries. The right mainstem bronchus appeared as a congenital diverticulum arising at the expected level of the true carina at T4. Distal to the carina, the left mainstem bronchus demonstrated a long segment of smooth, moderate stenosis. The displaced right mainstem bronchus originated from the left mainstem bronchus at the T6–T7 level. This anomalous airway trifurcated to supply the three-lobed right lung. The right lung was, however, hypoplastic in relation to the left. The left lung was bilobed with conventional branching of the upper and lower lobe bronchi (Figs 1 and 2).

Figure 1. Twenty-year-old male patient presenting with chronic dyspnea resistant to balloon dilation.3D volume rendered reformat of the airway and lungs demonstrates a hypoplastic right lung, congenital diverticulum representing the right mainstem bronchus and type 2 bridging bronchus arising from the left mainstem bronchus at the splayed pseudo-carina.

Figure 2. Twenty-year-old male patient presenting with chronic dyspnea. Axial contrast enhanced CT image demonstrates a left pulmonary artery sling posterior to the airway. In left pulmonary artery sling, the left pulmonary artery arises anomalously from the right pulmonary artery and travels in between the trachea and oesophagus before branching to supply the left lung.
This was associated with a left pulmonary artery sling (left pulmonary artery arising from the posterior aspect of the right pulmonary artery and coursing posterior to the pseudo-carina and anterior to the oesophagus).
These imaging findings are consistent with a type 2 bridging bronchus supplying the entire right lung with stenosis of the left mainstem bronchus and associated left pulmonary artery sling.
Discussion
Airway development, and therefore related anomalies, occur early in gestation starting from 24 days to approximately 17 weeks.Reference Chassagnon, Morel, Carpentier, Le Pointe and Sirinelli3 The anomalous airways can be either supernumerary or displaced bronchi.Reference Chassagnon, Morel, Carpentier, Le Pointe and Sirinelli3 A supernumerary bronchus co-exists with the normal patent bronchus and may either supply pneumatised lung or be blind-ended, whereas a displaced bronchus replaces a normal anatomical airway.Reference Chaddha, Chang and Lee2,Reference Chassagnon, Morel, Carpentier, Le Pointe and Sirinelli3
Several basic principles of airway anatomy exist. In general, the trachea is a 10–12-cm tube with incomplete cartilaginous rings bifurcating at the T4–T5 thoracic vertebral level.Reference Chaddha, Chang and Lee2,Reference Chassagnon, Morel, Carpentier, Le Pointe and Sirinelli3 The mainstem bronchi are wider and shorter.Reference Chaddha, Chang and Lee2 Normal situs bronchial anatomy includes a right eparterial bronchus and left hyparterial bronchus.Reference Chaddha, Chang and Lee2–Reference Subha, Gail, Alla Godelman and Spindola-Franco4
Some of the common congenital airway anomalies are tracheal and accessory cardiac bronchi, both of which are widely discussed in literature. However, in this case report, we are presenting a much rarer entity: a type 2 bridging bronchus supplying the entire right lung with an associated left pulmonary artery sling in an symptomatic adult male patient.
A bridging bronchus is diagnosed when all or part of the right lung is aerated by a displaced bronchus arising from the left mainstem bronchus.Reference Chaddha, Chang and Lee2,Reference Chassagnon, Morel, Carpentier, Le Pointe and Sirinelli3,Reference Westin and Kapalczynski5 There are two types of bridging bronchi.Reference Chassagnon, Morel, Carpentier, Le Pointe and Sirinelli3 In type 1, the right upper lobe bronchus arises from the trachea, whereas the right bronchus intermedius arises from the medial wall of the left mainstem bronchus as a bridging bronchus.Reference Chaddha, Chang and Lee2,Reference Chassagnon, Morel, Carpentier, Le Pointe and Sirinelli3 In type 2, the right mainstem bronchus is a blind-ended congenital tracheal diverticulum at the carina and the right lung is entirely aerated by a bridging bronchus arising from the medial wall of the left mainstem bronchus.Reference Chaddha, Chang and Lee2,Reference Chassagnon, Morel, Carpentier, Le Pointe and Sirinelli3 The origin of the bridging bronchus is at the level of T6–T7 (lower than the normal level of the carina at T4) and termed the pseudo-carina.Reference Chassagnon, Morel, Carpentier, Le Pointe and Sirinelli3
Bridging bronchus anomalies are very commonly associated with a left pulmonary artery sling, in up to 27% of type 1 cases and in up to 57% of type 2 cases.Reference Chassagnon, Morel, Carpentier, Le Pointe and Sirinelli3,Reference Westin and Kapalczynski5 An alternative classification scheme exists that describes the arterial sling as the main anomaly classified into types 1 and 2 depending on the presence or absence of a bridging bronchus.Reference Westin and Kapalczynski5 The presence of complete tracheal rings is an important finding in patients with type 2 pulmonary slings, termed the “ring-sling” complex.Reference Lee, Kim and Lee6 The tracheal rings with associated stenosis drive the patients’ ongoing shortness of breath with variable severity and can be fatal in early infancy.Reference Lee, Kim and Lee6 The gold standard for diagnosis is bronchoscopy, where a stenotic trachea and absence of the posterior membrane are seen.Reference Westin and Kapalczynski5 In the presence of complete cartilaginous tracheal rings, treatment options may expand to include tracheal resection or autograft rather than isolated bronchoscopic dilation depending on severity.Reference Westin and Kapalczynski5,Reference Lee, Kim and Lee6 In our case, the patient was found to have complete bronchial cartilage rings on bronchoscopy, but the thoracic surgeon decided to attempt bronchoscopic dilatation prior to surgery in the hopes that this would provide symptomatic relief.
Patients with bridging bronchi can present with wheezing, respiratory distress, or poor feeding, often in infancy.Reference Chassagnon, Morel, Carpentier, Le Pointe and Sirinelli3,Reference Westin and Kapalczynski5 Both cross-sectional assessment and bronchoscopy are usually warranted prior to treatment.Reference Chassagnon, Morel, Carpentier, Le Pointe and Sirinelli3
Conclusion
In the presented case, we demonstrate the expected findings of a type 2 bridging bronchus:
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The right mainstem bronchus is a blind diverticulum at T4.
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The splayed pseudo-carina is at T6–T7.
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The left mainstem bronchus is elongated and narrowed.
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The right lung is mildly hypoplastic.
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A left pulmonary artery sling is present.
Unfortunately, despite balloon dilatation, our patient remained symptomatic with recurrent dyspnea.
Acknowledgements
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Financial support
This paper received no specific grant from any funding agency, commercial or not-for-profit sectors.
Conflicts of interest
None.
Ethical standards
Not applicable.