You are on page 1of 4

Postgraduate Education Corner

PULMONARY, CRITICAL CARE, AND SLEEP PEARLS


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