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Common Congenital Anomalies of the Central Airways in AdultsCongenital Anomalies of the Central Airways FREE TO VIEW

Atul C. Mehta, MD, FCCP; Tany Thaniyavarn, MD; Michael Ghobrial, MD; Danai Khemasuwan, MD, MBA
Author and Funding Information

From the Department of Pulmonary Medicine (Dr Mehta), Respiratory Institute, Cleveland Clinic, Cleveland, OH; the Department of Pulmonary, Allergy, and Critical Care Medicine (Dr Thaniyavarn), Emory University, Atlanta, GA; the Internal Medicine Department (Dr Ghobrial), Fairview Hospital, a Cleveland Clinic Hospital, Cleveland, OH; and the Interventional Pulmonary Medicine (Dr Khemasuwan), Henry Ford Hospital, Detroit, MI.

CORRESPONDENCE TO: Atul C. Mehta, MD, FCCP, Pulmonary, Allergy and Critical Care Medicine, Respiratory Institute, Cleveland Clinic Foundation, Cleveland, OH 44195; e-mail: mehtaa1@ccf.org


Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2015;148(1):274-287. doi:10.1378/chest.14-1788
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The knowledge of airway anatomy is the most fundamental requirement of every bronchoscopist. There are numerous and frequent anatomic variations of the central airways making the examination unique for every individual. It is imperative for every bronchoscopist to be fully cognizant of the common congenital anomalies involving the central airways. Proper identification and reporting of these findings are a matter of the utmost importance, especially when surgical options in a patient with lung cancer or lung transplantation is under consideration. This article focuses on the congenital anomalies of central airway encountered among adults. Each of these anatomic variations has a characteristic appearance, yet requires bronchoscopic acumen for their identification. This review provides a comprehensive description of these anomalies and highlights their clinical implications.

Figures in this Article

Flexible bronchoscopy is the most commonly performed diagnostic procedure by the pulmonologist. It is estimated that > 500,000 bronchoscopies are performed annually in the United States.1 Thorough knowledge of the airway anatomy is the fundamental requirement of every bronchoscopist. Anatomic variations involving the central airways are numerous and frequent, making the airway examination unique for every individual (Figs 1A, 1B). Most anomalies rarely produce any symptoms and are often encountered during adulthood, when the procedure is being performed for an unrelated indication.2 It is imperative for every bronchoscopist to be fully cognizant of these anomalies, especially when advanced diagnostic or therapeutic procedures are under consideration. Accurate identification and reporting of these findings are essential, especially while performing electromagnetic navigation or stent placement. Proper inspection of the airways is also crucial among the donors as well as the recipients of lung transplantation. Despite increasing use of the CT imaging, these anomalies are seldom confirmed without bronchoscopy. Each of these anomalies has a characteristic appearance, yet requires added skills and experience for their recognition. This review provides a description of common congenital anomalies of the central airways among adults, including their epidemiology, clinical relevance, and management, if necessary. The review does not include anomalies that are strictly limited to the pediatric age group and those not compatible with life.

Figure Jump LinkFigure 1 –  A, Sub superior segment of right lower lobe. B, Trifurcation of lingula. C, Hemangioma involving the subglottic trachea. D, Tracheal bronchus: note an anomalous opening arising from the right tracheal wall above the main carina (arrow). E, Schematic depicting variations of the tracheal bronchus.Grahic Jump Location
Laryngeal Hemangioma

Laryngeal hemangiomas (LGHs) are classified into a pediatric and an adult form. The incidence of the former is approximately 1.5% of all anomalies of the larynx.3 The incidence in adults, however, is uncertain. LGHs can present at any age with vague symptoms such as hoarseness, cough, dyspnea, hemoptysis, and dysphagia. The latter was the most common complaint in a series by Kramer and Yankauer.4 LGH is usually supraglottic in adults; in children, however, the lesion involves the subglottic area in association with cutaneous lesions.3,5

The diagnosis of LGH in adults is challenging because of its nonspecific presentation and rarity. A high index of suspicion with a history of poorly controlled asthma and/or a finding of soft tissue density in the subglottic area on a CT scan raises the suspicion. Diagnosis is confirmed by bronchoscopy revealing a unilateral, bluish, smooth, and soft lesion involving either the supraglottic or subglottic region (Fig 1C).3,6

Management of LGH is variable. In the pediatric form, a spontaneous resolution is possible; hence, the treatment is conservative with goals of preserving laryngeal function.6 In adults, the treatment is individualized based on the size, location, symptoms, and the experience of the clinician.7,8 The options include systemic steroids, laser photocoagulation, intralesional steroid injection, and surgical excision. On occasion, a tracheostomy may be required. Laser therapy does not affect the time to spontaneous resolution of LGH. Systemic steroid may reduce the size of the lesion, yet the benefits may not outweigh the side effects.6

Tracheal Bronchus

The right and the left upper lobe bronchi arise from the respective mainstems in humans. The right upper lobe bronchus arises from the trachea and is a normal endobronchial finding among pigs and other ruminant mammals.9,10 However, the conformation is considered anomalous in humans when an airway segment arises from the trachea and supplies either an entire or a portion of the upper lobe. This anomaly is commonly referred as “tracheal bronchus,” “pig’s bronchus,” “bronchus suis,” “trifurcation of the carina,” or “bronchial trifurcation.”2

The tracheal bronchus refers to any anomaly involving the trachea, the carina, or the main bronchus supplying the upper lobe territory.9,10 The true tracheal bronchus, however, is any bronchus originating from the trachea within 2 to 6 cm from the carina, usually at the junction of the mid and the distal one-third of the trachea (Fig 1D).5,11 In a case series by Ghaye and colleagues,9 tracheal bronchus accounted for 7.5% of all tracheobronchial anomalies. In this series of 35 cases, 69% of tracheal bronchus originated from the main bronchus, 23% from the trachea, and 8% from the carina. The malformation has an overall prevalence of 0.1% to 5%: 0.1% to 2% for the right and 0.3% to 1% for the left.9 Tracheal bronchus is known to coexist with other congenital or genetic conditions, including Down syndrome, VACTERL syndrome (vertebral anomalies, anal atresia, cardiac defects, tracheoesophageal fistula and/or esophageal atresia, renal and radial anomalies, and limb defects), congenital cystic adenomatoid malformation, and congenital heart diseases.1114 Tracheal bronchus is “supernumerary” if it coexists with a normal branching of the upper lobe (Figs 1E, 2A, 2B). On the other hand, if it aerates the upper lobe segment(s) in the absence of one of its normal branches, is categorized as “displaced bronchus,” the latter being a more common.9,10

Figure Jump LinkFigure 2 –  A, An axial CT scan view shows the take-off of the tracheal bronchus into the apical segment of the right upper lobe (black arrow). B, A coronal view depicting tracheal bronchus (white arrow).Grahic Jump Location

Tracheal bronchus is usually asymptomatic and detected incidentally on chest imaging or bronchoscopy performed for unrelated reasons.9,15,16 However, it can become symptomatic if its drainage is impaired or it is associated with other anomalies.9,10 Patients may present with stridor, persistent cough, wheezing, hemoptysis, respiratory distress, upper lobe atelectasis, bronchiectasis, recurrent pneumonia, focal emphysema, or a thoracic mass.9,10,1517 There have been a few cases of neoplasms arising from the tracheal bronchus; however, this association seems to be a coincidence.18 If inadvertently intubated, the patient may present with hypoxemia, atelectasis, and/or air trapping.19 If it remains unrecognized, ipsilateral pneumothorax, atelectasis, or both may develop. Patients with persistent upper lobe atelectasis after intubation should prompt clinicians to consider this anomaly.17,20 Based on its location, the tracheal bronchus should be distinguished from the tracheoesophageal fistula (TEF), the latter arising from the posterior wall of the trachea.10

Asymptomatic patients with tracheal bronchus are managed conservatively. However, in a symptomatic patient, surgical resection may be required.15 To avoid complications of implanting the tracheal bronchus altogether, a segmentectomy of the area supplied by the tracheal bronchus in the donor lung is needed in cases of lung or heart-lung transplantation.21

Tracheal Diverticulum

Rokitansky first described the tracheal diverticulum in 1838.22 This anomaly is regarded as an anatomic variation of the tracheal bronchus. It is characterized by its narrow neck and a thick wall that consists of smooth muscles and a cartilage.23 It is also referred to as “tracheal pouch” or a “tracheocele.”22,24,25 It is more common in men and usually right sided. It arises 4 to 5 cm below the vocal cords (Fig 3A).23,26 Embryologic origin of the tracheal diverticulum involves cartilage defect or abnormal endodermal differentiation during the development of the posterior wall.27,28 Tracheal diverticulum could represent an aborted supernumerary bronchus or a high division of the primary lung bud. Its incidence in adults is unknown but ranges from 0.3% to 1% in the pediatric population.22,23,26

Figure Jump LinkFigure 3 –  A, Tracheal diverticulum: note a blind pouch arising from the right tracheal wall above the carina (arrow). B, Tracheoesophageal fistula: note an anomalous opening involving the posterior tracheal wall of the upper one-third of the trachea (arrow). C, Multiple complete tracheal rings (stove-pipe trachea). Note absence of posterior membranous wall. D, Pulmonary artery sling in a patient with complete tracheal ring. E, Axillary segment of the right upper lobe (arrow). F, Quadrivial right upper lobe bronchus. MC = main carina.Grahic Jump Location

In contrast to its congenital counterpart, acquired tracheal diverticulum from trauma or following TEF repair is a mucosal herniation through a weak point in the tracheal wall, thus lacking cartilage and smooth muscles (pseudodiverticulum) (Video 1).23,24 It has a thinner wall and is usually larger than the congenital form. It arises laterally between the cartilaginous rings or through the trachealis muscle at the level of the thoracic inlet.23,26,29,30 Because of the lack of esophageal support, the majority of acquired diverticula arise in the right paratracheal region. It is usually found at the level of the second thoracic vertebra (T2), which is the weakest point of the trachea where the extrathoracic trachea joins its intrathoracic counterpart.29,31 Multiple acquired tracheal diverticula are seen in association with Mounier-Kuhn syndrome.23

Video 1

The communication between tracheal diverticulum and the main trachea may not always be apparent on CT chest scan, and, thus, this condition must be distinguished from a paratracheal air-filled cyst by bronchoscopic examination.29,31 Most cases are asymptomatic and found incidentally.32 However, it may serve as a secretion reservoir causing pulmonary soiling, leading to chronic cough, dyspnea, or recurrent infection.22,23,30 On occasion, it may produce variable extrathoracic upper airway obstruction.33 It may exert a mass effect on adjacent organs causing globus pharyngeus29,31 or recurrent laryngeal nerve paralysis26 or present solely as a neck swelling.34 Occasionally, it can complicate the endotracheal intubation, which may lead to ventilatory difficulty25,35 or pneumomediastinum.36 Most tracheal diverticula produce minimal symptoms, yet occasionally they may require tracheal reconstruction, performed by either transthoracic, transcervical, or endoscopic approach.24 Surgical approach is preferred in the younger age group, whereas conservative management is preferred in elderly patients or patients with comorbidities.34

Tracheobronchial-Esophageal Fistula

Tracheobronchial-esophageal fistula (TBEF) is a communication between either the trachea or the bronchus and the esophagus. TBEF is either acquired or congenital in origin. An acquired form is either malignant or benign in nature. Trauma and infections account for most benign causes.37 On the other hand, congenital TBEF is a common anomaly of the respiratory tract with an incidence of approximately one in 3,500 live births.38 Congenital TEFs have five different types (Table 1).39 Similarly, congenital bronchoesophageal fistulas (BEFs) are of four types (Table 2).40 The BEF type II is the most prevalent, comprising 90% of cases in the largest series reported by Risher and colleages.41 Congenital TBEF rarely presents in adulthood, as it is often associated with other cardiopulmonary anomalies.42,43 The symptoms of TBEF presenting during adulthood include coughing (96%) and frequent respiratory infections (56%).44 Hemoptysis and halitosis are also common. Some patients may exhibit nonspecific GI symptoms. The duration of symptoms ranges from 6 months to 50 years before the confirmation of the diagnosis.41,45 There are two hypotheses explaining the delay in onset of symptoms from TBEF. First, there may be a membrane or a proximal fold of esophageal mucosa (flap valve) that becomes less mobile and less occlusive with age. Second, a fistulous tract running upward from the esophagus to the bronchus may close during swallowing, rarely causing aspiration.40,41,46 A spasm of the smooth muscle in the wall of the fistula may also aid in masking the presentation.47 However, none of these hypotheses are well supported.

Table Graphic Jump Location
TABLE 1 ]  E. C. Vogt Classification of Congenital Tracheoesophageal Fistulas39
Table Graphic Jump Location
TABLE 2 ]  Braimbridge and Keith’s40 Classification of Congenital Bronchoesophageal Fistulas

Despite the benign nature of the anomaly, it could lead to fatal complications if left untreated.42,47 A high index of suspicion is required to suspect TBEF in patients with repeated pulmonary infections and/or coughing spells associated with ingestion. The bronchoscopic findings depend upon the size and the location of the fistula (Fig 3B).48 A small TBEF covered by the airway mucosa may remain undetected even by experienced eyes, and an injection of methylene blue into the esophagus or radiographic examinations using contrast material may be required.

Thoracotomy for fistulectomy is the traditional surgical procedure for most cases of symptomatic TBEF.42,45,46 Fistulectomy with simple closure of the openings in both the organs is required. This has been associated with marked improvement in pulmonary function and disappearance of radiographic abnormalities.49 There are several other surgical options for the repair, including direct closure of the tracheal and esophageal defects with or without a muscle flap, tracheal resection and anastomosis with primary esophageal closure, tracheal closure with an esophageal or synthetic patch, and esophageal diversion.50,51 Nonsurgical approaches, such as the use of a self-expanding esophageal metal stent and endoscopic glue, have also been advocated.52

Congenital Tracheal Stenosis

Congenital tracheal stenosis is defined as a reduction of tracheal luminal diameter by > 50% of the remaining normal trachea.5355 It is categorized as a long-segment stenosis when the stenotic portion is > 50% of tracheal length.55 Congenital tracheal stenosis is subdivided into generalized hypoplasia, funnel-like narrowing, and segmental stenosis.56 Congenital tracheal stenosis may also be from the complete tracheal rings or from external compression.

The incidence of congenital tracheal stenosis in adults is unknown, but it is rare. Tracheal stenosis can be asymptomatic and discovered unexpectedly as a cause of difficult intubation57,58 or during a chest radiograph performed for unrelated reasons.59,60 In symptomatic cases, it can manifest as chronic dyspnea,54 recurrent infections,61 or severe or difficult-to-control asthma.61 Interestingly, most of the case reports in adults are women.62 The diagnosis is suspected based on a CT scan and confirmed with a bronchoscopic examination. Management of symptomatic congenital tracheal stenosis in adults is still controversial, owing to its rarity. A short-segment tracheal stenosis is managed with tracheal reconstruction, whereas a slide tracheoplasty is more preferable for a long-segment counterpart.62

Complete Tracheal Rings

With complete tracheal rings, the normal C-shaped tracheal cartilages are fused posteriorly, replacing normal “posterior pars membranacea.” Long tracheal stenosis from complete tracheal rings, referred as “stove-pipe” trachea, is a life-threatening condition with difficult management and a high mortality rate.53,54 It usually presents in the neonate or infant with severe respiratory distress (Fig 3C).6365 There is a strong association between vascular anomalies such as pulmonary artery sling or patent ductus arteriosus and complete tracheal rings, such that the discovery of one is an indication to search for the other.63,65

The condition is occasionally encountered among adults presenting as asthma mimics.53,60 A high index of suspicion is required to diagnose this condition, especially among patients with a vascular sling and refractory respiratory symptoms (Fig 3D).54 Diagnosis of complete tracheal rings can be suspected on a CT scan. The additional benefits of the CT scan in such high-risk patients is the ability to diagnose other cardiopulmonary anomalies. A virtual bronchoscopy can provide anatomic information from beyond the site of obstruction if the bronchoscopic examination is contraindicated.54,66

The treatment of complete tracheal ring is surgery, with selection of the procedure being based on the length of stenotic segment and the age of the patient.67,68 In general, asymptomatic patients or those with minimal symptoms are managed conservatively.69 On the other hand, for symptomatic cases with short-segment stenosis, tracheal reconstruction is preferred.6870 In adults with long-segment stenosis, slide tracheoplasty is the procedure of choice. However, tracheal autograft, rib grafting, and use of a pericardial patch have also emerged as possible alternatives.71

Although balloon bronchoplasty and rigid bronchoscopic dilation have been used successfully in the treatment of tracheobronchial stenosis, their use in the management of complete tracheal rings is controversial because of the potential for partial or a complete rupture of the tracheal rings resulting in related complications.72,73

Congenital Tracheobronchomalacia

Congenital tracheobronchomalacia (TBM) arises from a weakness of the tracheobronchial wall and cartilage, which leads to airway collapse during expiration. In adults, TBM is caused by either altered structures of the airway wall or acquired secondary factors (eg, inflammatory response).74

Patients with congenital tracheobronchomalacia typically present with nonspecific respiratory complaints, including dyspnea and chronic cough. Spirometry may show a rapid decline of expiratory flow after a peak related to the collapse of the central airway. However, these findings are not specific.75 One of the gold standards in diagnosis of TBM is bronchoscopy. Greater than 50% reduction in airway diameter during the expiratory phase confirms the diagnosis of TBM. An end-inspiratory and continuous dynamic expiratory CT scan is also used to detect TBM. The sensitivity of CT scan to diagnose TBM is 97% compared with the gold standard, bronchoscopy.76 Airway stenting can lead to symptomatic improvement. However, stent migration is a common complication.75 Airway stenting is usually used for a trial to identify those patients who are likely to benefit from tracheopexy.75

Axillary Bronchus

Axillary bronchus is a supernumerary bronchus supplying the lateral or axillary segment of the right or the left upper lobe, with the former being more common. The subsegmental type of the axillary segment is more common than the segmental type, which usually arises adjacent to the anterior segment of the right upper lobe (Fig 3E).77 The true incidence of the axillary bronchus is unknown; however, it is estimated that 10% of the population will have this supernumerary bronchus.78 Another variant of the axillary bronchus, a “quadrivial branching of the right upper lobe,” may be mistaken for an axillary bronchus.79 The quadrivial branching is found in 5.3% to 16% of all individuals (Fig 3F).79,80 Diseases of the axillary segment have distinct characteristics on the lateral chest radiograph of quadrangular-shaped opacity, which is bordered by both minor and major fissures (Fig 4). Awareness related to this anomaly is very helpful if a transbronchial biopsy is required from the corresponding axillary segment of the upper lobe.81

Figure Jump LinkFigure 4 –  A, Lateral view chest radiograph shows a quadrangular-shaped opacity. B, Axillary bronchus as seen on the CT scan of the chest (arrow).Grahic Jump Location
Accessory Cardiac Bronchus

In 1946, Brock described this anomaly as a supernumerary bronchus that originated from the medial wall of the right main bronchus or, to a lesser frequency, from a bronchus intermedius (Fig 5A).82 Accessory cardiac bronchus (ACB) arises proximal to the origin of the right middle lobe and the superior segmental bronchus.83 Eventually, it progresses medially and caudally toward the heart. Its incidence in the general population is around 0.08% to 0.5%, with a male to female ratio of 2.8:1.8284 However, it could be found with a much higher incidence (16%) among patients with other anomalies, such as tracheal bronchus, bronchiectasis, or double accessory cardiac bronchus.82,84 ACB can terminate in three different ways: blind ending, ventilated lobulus, or vestigial parenchyma. The majority of ACBs terminate into a blind extremity.82 If it terminates as a vestigial parenchyma, it may resemble a mass or a hypervascular lymph node on the CT scan of the chest.83 When it terminates as a ventilated lobule, it is located at the azygoesophageal recess demarcated by an abnormal fissure from the right lower lobe (Fig 6).82,83 Mean length of this abnormal bronchus is 12 mm (4.2-23.4 mm) with a mean diameter of 8.7 mm (4.0-13.8 mm).85 The shorter the accessory cardiac bronchus, the more likely it is to end as a blind pouch.84,85 If > 1 cm it may be associated with retained secretion resulting in recurrent infections.86 Its proximity to the right middle lobe accounts for frequent middle lobe infections; hence, its presence should be considered in a situation of difficult-to-treat middle lobe syndrome.83,86 Most cases are asymptomatic; however, superinfection, tumor, or aspergilloma may develop within the ACB and result in chronic cough, hemoptysis, or empyema.85,87,88

Figure Jump LinkFigure 5 –  A, ACB: note an anomalous opening arising from the medial wall of the right main bronchus. RML and lower lobe bronchi are visualized distally. B, Trifurcation of the left main bronchus. Note LUL, LIN, and LLL bronchi, all arising simultaneously. C, Bronchoscopic examination revealing absence of superior segment of the left lower lobe. D, Note the CT scan of the chest revealing hyperlucent superior segment of the left lower lobe. E, BB originating in the LMB crossed the mediastinum and provided the right lower and middle lobe. ACB = accessory cardiac bronchus; BB = bridging bronchus; BiT = bifurcatio tracheae; LIN = lingular; LLL = lower lobe; LMB = left mainstem bronchus; LUL = left upper lobe; RML = right middle lobe; RUL = right upper lobe bronchus; TS = tracheal stenosis.Grahic Jump Location
Figure Jump LinkFigure 6 –  A, B, Accessory cardiac bronchus (arrows) in axial (A) and coronal (B) CT scan views. C, A ventilated lobule (arrow) located at the azygoesophageal recess and demarcated by an abnormal fissure. (Image provided courtesy of Dr David Naidich.)Grahic Jump Location
Trifurcation of the Left Main Bronchus

Doshi and Mehta89 were the first to provide a detailed description of the left main bronchus (LMB) trifurcation in 2000. The incidence of this anomaly is unknown. In a bronchoscopy series by Beder and colleagues,2 475 of 1,114 patients were found to have at least one tracheobronchial variation; trifurcation of the LMB accounted for 0.1%. This anomaly is regarded as a displaced prehyparterial type of tracheal bronchus.90 Theoretically, it is possible that the trifurcation of the LMB is a type of left tracheal bronchus. It should be differentiated from the double-right-lung type of bronchial isomerism, which is a rare anatomic variation (situs solitus) consisting of a left lung that is identical to the right at the bronchial and lobar levels.91 This type of anatomic variation is usually associated with congenital asplenia and impaired immunity. The trifurcation of the LMB produces no symptoms and is usually an incidental finding during a bronchoscopic examination performed for an unrelated indication (Fig 5B).92

Bronchial Atresia

Ramsay93 first described this congenital anomaly in 1953 as an interruption of lobar, segmental, or subsegmental bronchus, with the segmental bronchus being most commonly involved.93,94 CT scan is the best diagnostic modality with the following characteristics: (1) mucocele, (2) occlusion of the bronchus central to the mucocele, and (3) hyperinflation of the lung distal to the atretic bronchus.95,96 The distal hyperinflation is believed to be as a result of collateral ventilation through the pores of Kohn, channels of Lambert, and interbronchiolar channels.97 Bronchoscopic findings can be normal or may show a blind-ending bronchus (Fig 5C). However, the latter without characteristic CT imaging findings may be a normal anatomic variation rather than an atretic bronchus.98 Bronchial atresia is most commonly found in the apicoposterior segmental bronchus of the left upper lobe, followed by segmental bronchus of the right upper, middle, and lower lobes, respectively (Fig 5D).99 It can be found in isolation or accompanying other malformations, namely congenital cystic adenomatoid malformation, bronchopulmonary sequestration, or congenital lobar emphysema.94,100 When a bronchial atresia is found in the lower lobe, especially the posterobasal segment of the left lower lobe, it is often associated with intralobar bronchopulmonary sequestration.94 The prevalence of this entity is unknown, but in a study performed on a male population its incidence was calculated to be 1.2 cases per 100,000.101 There is no sex predilection.97 With widespread in utero ultrasound screening, bronchial atresia has become a more commonly identified lesion, which could present as a lung mass.94 In adults, bronchial atresia is usually diagnosed in the second to fourth decade of life, but it can be found in the elderly as well.97 It is mostly benign and asymptomatic; however, it can manifest with chest pain, wheezing, dyspnea, recurrent pulmonary infection, lung or hilar mass, or as a cavitary lesion.95,97,101 The treatment is customized according to the symptoms.

Esophageal Bronchus

Esophageal bronchus is an abnormal communication between lung tissue and the esophagus. Bronchopulmonary foregut malformations include a variety of malformations, such as intralobar and extralobar pulmonary sequestration, foregut duplication cyst, and diverticula from the GI tract or the respiratory tree.102,103 At the embryogenic stage, if the lung bud migrates caudally along with the growing esophagus104 it could lead to the formation of the esophageal bronchus. The most common site of communication is the lower esophagus.105,106 Esophageal bronchus is commonly right sided, whereas pulmonary sequestration usually occurs on the left.107 There are no reports on the incidence of this anomaly, but it is more common among female patients, with a ratio of 1.5:1. The most common presentation is recurrent chest infections, which is similar to TBEF.108 An esophagogram could be diagnostic in patients with recurrent refractory chest infections suspected to have this condition. Thoracotomy and stapling of the communicating tract is the current standard treatment offered to patients with esophageal bronchus.103

Bridging Bronchus

Bridging bronchus is often associated with a sling left pulmonary artery.109 This anomaly is extremely rare and is usually found in infants. It can present with wheezing in an infant and can be incidentally found during bronchoscopic examination in an adult. A bridging bronchus originates from medial wall of left mainstem bronchus. Its course crossed the mediastinum and supplied the right middle lobe and right lower lobe (Figure 5E).110 Surgical correction may be needed for recurrent atelectasis of the right middle lobe or right lower lobe.109

The knowledge of normal anatomy of the central airway and its common variations is a fundamental skill of every pulmonologists. The clinical presentation is usually nonspecific, and the anomalies may be difficult to diagnose unless direct visualization is performed. Most frequently these anomalies are incidentally found during a bronchoscopic examination performed for unrelated indications. The summary of these congenital anomalies of central airways is illustrated in Table 3. The complete description and documentation are very important for future interventions, if ever required.

Table Graphic Jump Location
TABLE 3 ]  Common Congenital Anomalies of the Central Airways in Adults

LMS = left mainstem; N/A = not available; RLL = right lower lobe; RML = right middle lobe; RUL = right upper lobe.

Financial/nonfinancial disclosures: The authors have reported to CHEST that no potential conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

Additional information: The Video can be found in the Multimedia section of the online article.

ACB

accessory cardiac bronchus

BEF

bronchoesophageal fistula

LGH

laryngeal hemangioma

LMB

left main bronchus

TBEF

tracheobronchial-esophageal fistula

TBM

tracheobronchomalacia

TEF

tracheoesophageal fistulas

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Figures

Figure Jump LinkFigure 1 –  A, Sub superior segment of right lower lobe. B, Trifurcation of lingula. C, Hemangioma involving the subglottic trachea. D, Tracheal bronchus: note an anomalous opening arising from the right tracheal wall above the main carina (arrow). E, Schematic depicting variations of the tracheal bronchus.Grahic Jump Location
Figure Jump LinkFigure 2 –  A, An axial CT scan view shows the take-off of the tracheal bronchus into the apical segment of the right upper lobe (black arrow). B, A coronal view depicting tracheal bronchus (white arrow).Grahic Jump Location
Figure Jump LinkFigure 3 –  A, Tracheal diverticulum: note a blind pouch arising from the right tracheal wall above the carina (arrow). B, Tracheoesophageal fistula: note an anomalous opening involving the posterior tracheal wall of the upper one-third of the trachea (arrow). C, Multiple complete tracheal rings (stove-pipe trachea). Note absence of posterior membranous wall. D, Pulmonary artery sling in a patient with complete tracheal ring. E, Axillary segment of the right upper lobe (arrow). F, Quadrivial right upper lobe bronchus. MC = main carina.Grahic Jump Location
Figure Jump LinkFigure 4 –  A, Lateral view chest radiograph shows a quadrangular-shaped opacity. B, Axillary bronchus as seen on the CT scan of the chest (arrow).Grahic Jump Location
Figure Jump LinkFigure 5 –  A, ACB: note an anomalous opening arising from the medial wall of the right main bronchus. RML and lower lobe bronchi are visualized distally. B, Trifurcation of the left main bronchus. Note LUL, LIN, and LLL bronchi, all arising simultaneously. C, Bronchoscopic examination revealing absence of superior segment of the left lower lobe. D, Note the CT scan of the chest revealing hyperlucent superior segment of the left lower lobe. E, BB originating in the LMB crossed the mediastinum and provided the right lower and middle lobe. ACB = accessory cardiac bronchus; BB = bridging bronchus; BiT = bifurcatio tracheae; LIN = lingular; LLL = lower lobe; LMB = left mainstem bronchus; LUL = left upper lobe; RML = right middle lobe; RUL = right upper lobe bronchus; TS = tracheal stenosis.Grahic Jump Location
Figure Jump LinkFigure 6 –  A, B, Accessory cardiac bronchus (arrows) in axial (A) and coronal (B) CT scan views. C, A ventilated lobule (arrow) located at the azygoesophageal recess and demarcated by an abnormal fissure. (Image provided courtesy of Dr David Naidich.)Grahic Jump Location

Tables

Table Graphic Jump Location
TABLE 1 ]  E. C. Vogt Classification of Congenital Tracheoesophageal Fistulas39
Table Graphic Jump Location
TABLE 2 ]  Braimbridge and Keith’s40 Classification of Congenital Bronchoesophageal Fistulas
Table Graphic Jump Location
TABLE 3 ]  Common Congenital Anomalies of the Central Airways in Adults

LMS = left mainstem; N/A = not available; RLL = right lower lobe; RML = right middle lobe; RUL = right upper lobe.

Video 1

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