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Postgraduate Education Corner: Pulmonary, Critical Care, and Sleep Pearls |

A 46-Year-Old Woman With Persistent Asthma and Lung MassesPersistent Asthma and Lung Masses FREE TO VIEW

Thomas Waring, MD; Danae Delivanis, MD; Electra Kaloudis, MD; Debapriya Datta, MD, FCCP
Author and Funding Information

From the Department of Pulmonary and Critical Care Medicine (Drs Waring and Datta), the Department of Internal Medicine (Dr Delivanis), and the Department of Radiology (Dr Kaloudis), University of Connecticut Health Center, Farmington, CT.

Correspondence to: Debapriya Datta, MD, FCCP, Division of Pulmonary and Critical Care Medicine, University of CT Health Center, 263 Farmington Ave, Farmington, CT 06030; e-mail: ddatta@uchc.edu


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


Chest. 2014;145(5):1166-1169. doi:10.1378/chest.13-2208
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Published online

A 46-year-old woman presented with difficulty breathing with exertion for the last few months. She had recently been diagnosed with asthma and was prescribed inhaled bronchodilators without significant improvement. She was using rescue bronchodilators three times a day. She denied fevers, chronic cough, hemoptysis, chest pain, nasal congestion, or postnasal drip. There was no history of nocturnal symptoms, heartburn, weight loss, or loss of appetite. She had previously never had a chest radiograph. Her past medical history was otherwise negative. She was using an albuterol metered-dose inhaler as needed. She did not smoke or use any illicit substances. She was unemployed and had no pets. Family history was unremarkable.

Physical Examination Findings

Physical examination revealed a healthy-appearing woman in no distress. Her pulse was 78 beats/min, regular; BP, 110/70 mm Hg; respiratory rate, 16 breaths/min; and oxygen saturation, 96% on room air. There was no pallor, icterus, or cyanosis, and no jugular venous distension, thyromegaly, or cervical lymphadenopathy. Lungs were clear to auscultation. No wheezing or rhonchi was present. Cardiac examination was normal. Abdominal examination revealed no tenderness, guarding, or hepatosplenomegaly. No pedal edema or clubbing was noted.

Diagnostic Studies

Hemoglobin, WBC count, platelet counts, and basic metabolic panel results were normal. Pulmonary function tests revealed no obstruction, with normal lung volumes and diffusion capacity. Chest radiograph was performed (Fig 1). To further evaluate the abnormalities on chest radiograph, a CT scan of the chest was performed (Figs 2, 3).

Figure Jump LinkFigure 1. Chest radiograph showing two circumscribed masses in the right upper lobe, close to the right hilum.Grahic Jump Location
Figure Jump LinkFigure 2. CT scan of the chest. Well-circumscribed mass in the perihilar region of the right lung. Focal emphysema is present distal to the mass.Grahic Jump Location
Figure Jump LinkFigure 3. CT scan of the chest. A tubular mass is seen near the hilum in the right upper lobe. Localized air trapping and hyperinflation are seen distal to the mass-like lesion.Grahic Jump Location
What is the diagnosis?
Diagnosis: Congenital bronchial atresia with mucocele

Congenital bronchial atresia (CBA) is a rare congenital tracheobronchial anomaly, characterized by a focal narrowing of a subsegmental, segmental, or lobar bronchus. It is the second most common congenital tracheobronchial anomaly after bronchopulmonary sequestration. The incidence of CBA is estimated to be 1.2 in 100,000. It predominantly affects men. Ages of reported cases have ranged from newborn to 67 years. The majority of cases are adolescents or young adults, with average age at diagnosis being 22 years. The cause remains uncertain; however, vascular insult or insufficiency during fetal development has been proposed as a pathogenesis for this abnormality. This affects the development of the segment of the bronchial tree arising from dorsal bud of the enteric mesenchyme, resulting in atresia. After birth, this atresia results in poststenotic dilatation of the affected bronchus, which terminates blindly and is not connected to the central airway. This develops into a mucus-filled cavity (mucocele) from mucus secretion without drainage.

The majority of patients are asymptomatic when discovered on imaging studies. Less than 30% present with recurrent respiratory tract infection. Patients usually present with cough, shortness of breath, or frequent respiratory infections. An association with asthma has been reported. On examination, there are no specific physical signs diagnostic of CBA. No thoracic cage deformity or asymmetry of chest wall has been reported. Respiratory symptoms are secondary to infection in mucocele resulting from atretic bronchi and compression of adjoining lung. The left upper lobe is most commonly affected (50% of cases), followed by right upper lobe (18%), left lower lobe (15%), right lower lobe (10%), and right middle lobe (7%).

Chest radiographs and CT scans usually show a mass-like opacity. Mucus-filled bronchus is seen as a rounded or tubular opacity near the hilum. The opacity may be dense and rounded, or a cyst with air-fluid levels, or tubular with branching and multiple lobulations. Usually a single lesion is seen; two or more lesions are uncommon. There is hyperinflation of the obstructed segment of contiguous lung, distal to the mucus-impacted obstructed bronchus, which manifests as localized air trapping and hyperlucency. This is due to ventilation occurring in the affected segment or lobe distal to the obstructed bronchi by interlobular microscopic channels, such as the channels of Lambert and pores of Kohn. These collateral airways aerate the alveoli, but exhalation is limited compared with inhalation, resulting in some air trapping and localized hyperinflation. This manifests as a localized hyperlucency, which is clearly evident on CT scan of the chest but difficult to appreciate on chest radiograph. Mass-like lesions are reported to be present in 80%, localized hyperlucency distal to the mass in 76%, and both features are present concurrently in 58%.

Previously, bronchography and bronchoscopy were recommended as part of the evaluation of CBA. However, these were not always diagnostic, and often patients ended up with surgical resections because of inability to diagnose these lesions by imaging, airway evaluation, or biopsy. Current-generation CT scanners provide a detailed sequential cross-sectional view of abnormal airways and also detect localized air trapping and hyperlucency, which is part of the diagnostic criteria for CBA. CT scan with contrast also helps rule out anomalies such as bronchopulmonary sequestration or bronchogenic cyst, which are differentials for CBA. Allergic bronchopulmonary aspergillosis results in mucoceles from mucoid impaction of dilated bronchi and is another differential for CBA. However, allergic bronchopulmonary aspergillosis can be differentiated from CBA by the presence of associated abnormalities in the lung, including central bronchiectasis, bronchial wall thickening, fleeting alveolar opacities, and tree-in-bud opacities.

At present, CBA is usually diagnosed by CT scan of the chest. Bronchography or bronchoscopy could show the involved atretic bronchial segment but may be nondiagnostic, depending on site of involvement. However, bronchoscopy should be performed to exclude tumor, foreign body, or stricture as a cause of bronchial obstruction. Tissue biopsy is not essential for diagnosis, if characteristic CT scan features are present. In the absence of characteristic CT scan features, tissue biopsy should be obtained. For increasing diagnostic yield, and obtaining adequate tissue, this should be done thoracoscopically. Histopathology of resected mass-like lesions in patients with CBA shows dilated small bronchi and bronchioles filled with mucus and adjoining alveolar distention/emphysema, with no evidence of malignancy.

Management of CBA is conservative. In asymptomatic patients incidentally found to have this condition, no interventions are necessary. Superimposed infections are treated with antibiotics. Surgery is generally reserved for patients with recurrent infections. Lobectomy was reported in 50% of cases in one series. The current standard of practice is surgical resection of involved segment or lobe only if the patient has recurrent infections.

The characteristic CT scan findings resulted in the diagnosis of CBA in this patient. The mass-like lesions in the right upper lobe represented mucoceles, caused by obstructed bronchi, which are dilated because of mucus impaction. CT scan of the chest (Fig 2) shows a well-circumscribed mass in the perihilar region of the right lung representing one mucocele. Figure 3 shows another tubular, perihilar mass representing a second mucocele due to mucus impaction resulting from atretic bronchus in the right upper lobe. Localized air trapping and hyperinflation of obstructed segment of lung manifested as increased lucency distal to the lesion is seen in both Figures 2 and 3. Based upon these characteristic chest CT scan findings, the diagnosis of CBA was made.

Clinical Course

The patient was informed of this new diagnosis and the need for regular pulmonary follow-up. She did not wish to undergo bronchoscopy as part of her evaluation. Her main complaint pertained to her inadequately controlled asthma, for which she was started on inhaled corticosteroids. This resulted in significant improvement in her symptoms. On follow-up 1 year later, the patient is doing well, without any sequelae so far.

  • 1. CBA is rare, but constitutes the second most common congenital tracheobronchial malformation, after bronchopulmonary sequestration.

  • 2. CBA should be suspected in patients with airway symptoms and mass-like lesion on CT scan of the chest, with localized hyperlucency distal to the lesion.

  • 3. Bronchoscopy should be performed to exclude acquired proximal bronchial obstruction by tumor, foreign body, or inflammatory stricture.

  • 4. If CT scan of the chest does not show characteristic findings, thoracoscopic biopsy should be performed to confirm the diagnosis.

  • 5. No interventions are necessary in asymptomatic patients with incidentally found lesion. Surgical excision should be reserved for patients with recurrent respiratory tract infections.

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.

Other contributions:CHEST worked with the authors to ensure that the Journal policies on patient consent to report information were met.

Ramsay BH, Byron FX. Mucocele, congenital bronchiectasis, and bronchiogenic cyst. J Thorac Surg. 1953;26(1):21-30. [PubMed]
 
Jederlinic PJ, Sicilian LS, Baigelman W, Gaensler EA. Congenital bronchial atresia. A report of 4 cases and a review of the literature. Medicine (Baltimore). 1987;66(1):73-83. [CrossRef] [PubMed]
 
Kinsella D, Sissons G, Williams MP. The radiological imaging of bronchial atresia. Br J Radiol. 1992;65(776):681-685. [CrossRef] [PubMed]
 
Mori M, Kidogawa H, Moritaka T, Ueda N, Furuya K, Shigematsu S. Bronchial atresia: report of a case and review of the literature. Surg Today. 1993;23(5):449-454. [CrossRef] [PubMed]
 
Ward S, Morcos SK. Congenital bronchial atresia—presentation of three cases and a pictorial review. Clin Radiol. 1999;54(3):144-148. [CrossRef] [PubMed]
 
Zylak CJ, Eyler WR, Spizarny DL, Stone CH. Developmental lung anomalies in the adult: radiologic-pathologic correlation. Radiographics. 2002;22(spec no):S25-S43. [CrossRef] [PubMed]
 
Cappeliez S, Lenoir S, Validire P, Gossot D. Total endoscopic lobectomy and segmentectomy for congenital bronchial atresia. Eur J Cardiothorac Surg. 2009;36(1):222–-224. [CrossRef] [PubMed]
 
Wang Y, Dai W, Sun Y, Chu X, Yang B, Zhao M. Congenital bronchial atresia: diagnosis and treatment. Int J Med Sci. 2012;9(3):207-212. [CrossRef] [PubMed]
 

Figures

Figure Jump LinkFigure 1. Chest radiograph showing two circumscribed masses in the right upper lobe, close to the right hilum.Grahic Jump Location
Figure Jump LinkFigure 2. CT scan of the chest. Well-circumscribed mass in the perihilar region of the right lung. Focal emphysema is present distal to the mass.Grahic Jump Location
Figure Jump LinkFigure 3. CT scan of the chest. A tubular mass is seen near the hilum in the right upper lobe. Localized air trapping and hyperinflation are seen distal to the mass-like lesion.Grahic Jump Location

Tables

Suggested Readings

Ramsay BH, Byron FX. Mucocele, congenital bronchiectasis, and bronchiogenic cyst. J Thorac Surg. 1953;26(1):21-30. [PubMed]
 
Jederlinic PJ, Sicilian LS, Baigelman W, Gaensler EA. Congenital bronchial atresia. A report of 4 cases and a review of the literature. Medicine (Baltimore). 1987;66(1):73-83. [CrossRef] [PubMed]
 
Kinsella D, Sissons G, Williams MP. The radiological imaging of bronchial atresia. Br J Radiol. 1992;65(776):681-685. [CrossRef] [PubMed]
 
Mori M, Kidogawa H, Moritaka T, Ueda N, Furuya K, Shigematsu S. Bronchial atresia: report of a case and review of the literature. Surg Today. 1993;23(5):449-454. [CrossRef] [PubMed]
 
Ward S, Morcos SK. Congenital bronchial atresia—presentation of three cases and a pictorial review. Clin Radiol. 1999;54(3):144-148. [CrossRef] [PubMed]
 
Zylak CJ, Eyler WR, Spizarny DL, Stone CH. Developmental lung anomalies in the adult: radiologic-pathologic correlation. Radiographics. 2002;22(spec no):S25-S43. [CrossRef] [PubMed]
 
Cappeliez S, Lenoir S, Validire P, Gossot D. Total endoscopic lobectomy and segmentectomy for congenital bronchial atresia. Eur J Cardiothorac Surg. 2009;36(1):222–-224. [CrossRef] [PubMed]
 
Wang Y, Dai W, Sun Y, Chu X, Yang B, Zhao M. Congenital bronchial atresia: diagnosis and treatment. Int J Med Sci. 2012;9(3):207-212. [CrossRef] [PubMed]
 
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