CHEST 1166 Postgraduate Education Corner CHEST 2014; 145 ( 5 ): 1166 1169 A 46-year-old woman presented with difculty breath- ing with exertion for the last few months. She had recently been diagnosed with asthma and was pre- scribed 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 med- ical 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, reg- ular; BP, 110/70 mm Hg; respiratory rate, 16 breaths/min; and oxy gen 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 nor- mal. 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 A 46-Year-Old Woman With Persistent Asthma and Lung Masses Thomas Waring , MD ; Danae Delivanis , MD ; Electra Kaloudis , MD ; and Debapriya Datta , MD, FCCP
Figure 1. Chest radiograph showing two circumscribed masses in the right upper lobe, close to the right hilum .
Figure 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. Downloaded From: http://journal.publications.chestnet.org/ by M Darwich on 10/15/2014 CHEST / 145 / 5 / MAY 2014 1167 journal.publications.chestnet.org function tests revealed no obstruction, with normal lung volumes and diffusion capacity. Chest radiograph was performed ( Fig 1 ). To further evaluate the abnor- malities on chest radiograph, a CT scan of the chest was performed ( Figs 2 , 3 ). What is the diagnosis?
Figure 3. CT scan of the chest. A tubular mass is seen near the hilum in the right upper lobe. Localized air trapping and hyperin- ation are seen distal to the mass-like lesion. Manuscript received September 17 , 2013 ; revision accepted October 14 , 2013 . Afliations: From the Department of Pulmonary and Critical Care Medicine (Drs Waring and Datta), the Department of Inter- nal Medicine (Dr Delivanis), and the Department of Radiology (Dr Kaloudis), University of Connecticut Health Center, Far- mington, 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 2014 American College of Chest Physicians. Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details. DOI: 10.1378/chest.13-2208 Downloaded From: http://journal.publications.chestnet.org/ by M Darwich on 10/15/2014 1168 Postgraduate Education Corner Diagnosis: Congenital bronchial atresia with mucocele Discussion Congenital bronchial atresia (CBA) is a rare con- geni tal 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 insuf- ciency during fetal development has been proposed as a pathogenesis for this abnormality. This affects the development of the segment of the bronchial tree aris ing 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-lled cavity (muco- cele) from mucus secretion without drainage. The majority of patients are asymptomatic when dis- covered on imaging studies. Less than 30% present with recurrent respiratory tract infection. Patients usu- ally present with cough, shortness of breath, or frequent respiratory infections. An association with asthma has been reported. On examination, there are no specic 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 compres- sion of adjoining lung. The left upper lobe is most com- monly 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-lled bronchus is seen as a rounded or tubular opacity near the hilum. The opacity may be dense and rounded, or a cyst with air-uid levels, or tubular with branching and multiple lobulations. Usually a single lesion is seen; two or more lesions are uncommon. There is hyperination 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 micro- scopic channels, such as the channels of Lambert and pores of Kohn. These collateral airways aerate the alveoli, but exhalation is limited compared with inha- lation, resulting in some air trapping and localized hyperination. This manifests as a localized hyperlu- cency, which is clearly evident on CT scan of the chest but difcult to appreciate on chest radiograph. Mass- like lesions are reported to be present in 80%, local- ized 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. How- ever, 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 hyper- lucency, which is part of the diagnostic criteria for CBA. CT scan with contrast also helps rule out anom- alies such as bronchopulmonary sequestration or bron- chogenic 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 bronchopul- monary aspergillosis can be differentiated from CBA by the presence of associated abnormalities in the lung, including central bronchiectasis, bronchial wall thickening, eeting 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 non- diagnostic, depending on site of involvement. How- ever, bronchoscopy should be performed to exclude tumor, foreign body, or stricture as a cause of bron- chial obstruction. Tissue biopsy is not essential for diag- nosis, 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 lled with mucus and adjoining alveolar distention/emphysema, with no evi- dence of malignancy. Management of CBA is conservative. In asymptom- atic patients incidentally found to have this condi- tion, no interventions are necessary. Superimposed infections are treated with antibiotics. Surgery is gen- erally reserved for patients with recurrent infections. Lobectomy was reported in 50% of cases in one series. The current standard of practice is surgical resec- tion of involved segment or lobe only if the patient has recurrent infections. The characteristic CT scan ndings 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 Downloaded From: http://journal.publications.chestnet.org/ by M Darwich on 10/15/2014 CHEST / 145 / 5 / MAY 2014 1169 journal.publications.chestnet.org shows another tubular, perihilar mass representing a second mucocele due to mucus impaction resulting from atretic bronchus in the right upper lobe. Local- ized air trapping and hyperination of obstructed seg- ment 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 ndings, 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 evalua- tion. Her main complaint pertained to her inadequately controlled asthma, for which she was started on inhaled corticosteroids. This resulted in signicant improve- ment in her symptoms. On follow-up 1 year later, the patient is doing well, without any sequelae so far. Clinical Pearls 1. CBA is rare, but constitutes the second most common congenital tracheobronchial malformation, after bronchopulmonary sequestration. 2. CBA should be suspected in patients with air- way 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, for- eign body, or inammatory stricture. 4. If CT scan of the chest does not show charac- teristic ndings, thoracoscopic biopsy should be per- formed to conrm the diagnosis. 5. No interventions are necessary in asymptom- atic patients with incidentally found lesion. Surgical excision should be reserved for patients with recurrent respiratory tract infections. Acknowledgments Financial/nonnancial disclosures: The authors have reported to CHEST that no potential conicts of interest exist with any companies/organizations whose products or services may be dis- cussed in this article . Other contributions: CHEST worked with the authors to ensure that the Journal policies on patient consent to report information were met. Suggested Readings Ramsay BH , Byron FX . Mucocele, congenital bronchiectasis, and bronchiogenic cyst . J Thorac Surg . 1953 ; 26 ( 1 ): 21 - 30 . Jederlinic PJ , Sicilian LS , Baigelman W , Gaensler EA . Congenital bronchial atresia. A report of 4 cases and a review of the litera- ture . Medicine (Baltimore) . 1987 ; 66 ( 1 ): 73 - 83 . Kinsella D , Sissons G , Williams MP . The radiological imaging of bronchial atresia . Br J Radiol . 1992 ; 65 ( 776 ): 681 - 685 . 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 . Ward S , Morcos SK . Congenital bronchial atresiapresentation of three cases and a pictorial review . Clin Radiol . 1999 ; 54 ( 3 ): 144 - 148 . Zylak CJ , Eyler WR , Spizarny DL , Stone CH . Developmental lung anomalies in the adult: radiologic-pathologic correlation . Radiographics . 2002 ; 22 ( spec no ): S25 - S43 . Cappeliez S , Lenoir S , Validire P, Gossot D . Total endoscopic lobec- tomy and segmentectomy for congenital bronchial atresia . Eur J Cardiothorac Surg . 2009 ; 36 ( 1 ): 222 224 . Wang Y , Dai W , Sun Y , Chu X , Yang B , Zhao M . Congenital bron- chial atresia: diagnosis and treatment . Int J Med Sci . 2012 ; 9 ( 3 ): 207 - 212 . Downloaded From: http://journal.publications.chestnet.org/ by M Darwich on 10/15/2014