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Chest Imaging and Pathology for Clinicians |

A 28-Year-Old Woman With Branching Opacity and Chest Pain FREE TO VIEW

Daniel D. Droukas, MD; Stephen C. Machnicki, MD
Author and Funding Information

Department of Diagnostic Radiology, Lenox Hill Hospital, New York, NY

CORRESPONDENCE TO: Daniel D. Droukas, MD, Department of Diagnostic Radiology, Lenox Hill Hospital, 100 East 77th St, New York, NY 10075


Copyright 2017, American College of Chest Physicians. All Rights Reserved.


Chest. 2017;151(4):e85-e89. doi:10.1016/j.chest.2016.12.022
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A 28-year-old female patient presented through her primary care physician with symptoms of atypical chest pain and chronic cough. Her pain was described as pleuritic and intermittently radiating to the right arm. Her medical history was significant for recurrent respiratory infections, gastritis, and a left ovarian cyst treated with ipsilateral salpingo-oophorectomy. She denied any history of smoking, known lung disease, or extrapulmonary infections.

Figures in this Article

Physical examination revealed an afebrile, well-nourished white woman in no acute distress. Her blood pressure was 128/85 mm Hg with a pulse of 74 beats/min and a respiratory rate of 16 breaths/min. Cardiovascular examination revealed a normal cardiac rhythm without murmur, rubs, or gallops. Lungs were clear to auscultation and percussion bilaterally. There was no evidence of recruitment of accessory muscles of respiration. Her extremities showed no cyanosis, clubbing, or edema. Arterial blood gas analysis on room air at rest revealed a pH of 7.34, Paco2 of 45 mm Hg, and Pao2 of 99 mm Hg. CBC count revealed a leukocytosis of 12,900 cells per mm3, and serum chemistry results were unremarkable. D-dimer values were not obtained.

Posteroanterior and lateral chest radiographs revealed a large tubular branching opacity overlying the right upper lobe (Fig 1). Contrast-enhanced CT scan showed this lesion to be nonenhancing and surrounded by a wedge-shaped area of hyperlucency pointing to the ipsilateral hilum (Fig 2). No lymphadenopathy was identified. Ventilation/perfusion scintigraphy showed virtually no ventilation or perfusion in the expanded posterior segment of the right upper lobe (Fig 3).

Figure 1
Figure Jump LinkFigure 1 A and B, Posteroanterior and lateral radiographs of the chest demonstrates a tubular branching opacity overlying the region of the right upper lobe (white arrows) with adjacent paucity of pulmonary vascular markings.Grahic Jump Location
Figure 2
Figure Jump LinkFigure 2 A, Contrast-enhanced axial CT image of the chest with lung windows shows a tubular, nonenhancing lesion (white arrow) surrounded by an area of wedge-shaped hyperlucency (curved white arrows) within the right upper lobe. B, Oblique maximum intensity projection with lung-windows better emphasizes the tubular branching nature of the lesion (white arrow) as well as narrowed vasculature within affected parenchyma. C, Coronal minimum intensity projection with lung windows emphasizes wedge-shaped peripheral hyperlucency secondary to air trapping (white arrows).Grahic Jump Location
Figure 3
Figure Jump LinkFigure 3 A, Planar ventilation scintigraphy following inhalation of technetium-99m-labeled DTPA (diethylene-triamine-pentaacetate) aerosol shows a wedge-shaped ventilation defect radiating from the right hilum (white arrow). B, Fused single-photon emission CT/CT scanning following the injection of 4 mCi of technetium-99m-labeled macroaggregated albumin shows an associated perfusion defect (white arrow), corroborating CT findings of regional oligemia within the affected lung parenchyma.Grahic Jump Location

The patient was referred to cardiothoracic surgery for posterior segmentectomy of the right upper lobe. Gross specimens revealed a soft, pliable mass surrounded by hyperexpanded pulmonary parenchyma. Bronchi proximal to the mass were narrow in caliber, whereas those more distal were dilated and with evidence of mucostasis. No discrete luminal connection was identified between proximal and distal bronchi. Microscopic analysis revealed dilated air spaces and mucostasis with evidence of chronic inflammation and peribronchovascular fibrosis (Fig 4).

Figure 4
Figure Jump LinkFigure 4 A, Section from the resected mass at low-power magnification shows bronchiolar dilation with areas of mucostasis (arrows). B, Intermediate magnification reveals peribronchial fibrosis (curved arrow) with lymphocytic infiltrate (arrow). C, High-power magnification emphasizes diffusely dilated alveolar air spaces. Two lymph nodes sent separately for review were benign (not shown).Grahic Jump Location

What is the diagnosis?

Diagnosis: Bronchial atresia, right upper lobe

Clinical Discussion

Bronchial atresia is a relatively rare defect characterized by focal interruption of a lobar, segmental, or subsegmental bronchus. This obstruction leads to peripheral mucus impaction with secondary hyperinflation of the distally obstructed lung parenchyma. The apicoposterior segment of the left upper lobe is most commonly affected, followed by the right upper, right middle, and right lower lobes, respectively. Few cases of multifocal disease have been reported.,

Although the etiology of bronchial atresia is not clearly defined, many investigators believe the defect to be congenital. Two leading developmental theories have been proposed. The first suggests a separation of distally dividing bronchial cells from the developing bronchial bud. This disconnected cell mass continues to develop normally, resulting in a normal branching pattern distally. The second theory suggests an in utero vascular insult leading to focal ischemia of the developing bronchial wall, causing short segmental atresia and obstruction to the distal bronchial tree.,

Generally, no luminal connection between the distal and proximal airways exists, although a thin fibrous band may sometimes bridge the two segments. Continued ciliary action within distal airways results in progressive mucus impaction, eventually leading to mucocele formation. The hyperinflation of distal lung parenchyma likely occurs due to expiratory air trapping through collateral pathways, including intra-alveolar pores of Kohn, bronchoalveolar channels of Lambert, and interbronchiolar channels.

A common developmental pathway was first proposed in 1975 linking a wide spectrum of congenital pulmonary anomalies to bronchial atresia, including congenital pulmonary airway malformation, bronchogenic cyst, bronchopulmonary sequestration, and congenital lobar emphysema. This pathway proposes a malformation sequence, in which an atretic bronchial segment early in gestation (4-6 weeks) predisposes to the formation of congenital pulmonary airway malformation or bronchogenic cyst. Later obstruction (16-18 weeks) is believed to favor the formation of bronchopulmonary sequestration or congenital lobar emphysema., This idea is supported by common histopathologic findings reported in pediatric patients.

Most cases of bronchial atresia are identified incidentally on chest radiographs in asymptomatic children or adults.,, Clinical manifestations of symptomatic patients may vary but include recurrent pulmonary infections, wheezing, atypical chest pain, and dyspnea. Asymptomatic patients may be followed up clinically and radiographically, with surgical resection of the affected segment or lobe indicated in those patients with recurrent pulmonary infections or when major clinical symptoms are present.

Radiologic Discussion

Radiographic findings are highly diagnostic and include a rounded, tubular, or branching opacity pointing toward the ipsilateral hilum with surrounding pulmonary hyperlucency. These opacities may contain air-fluid levels when complicated by superimposed infection and can be seen with simultaneous hyperinflation in 57% to 83% of cases., Nonenhanced CT scans usually demonstrate low attenuation of impacted secretions (–5 to 25 HU). Regional oligemia in hyperlucent areas is suggested by reduced vessel diameters due to intrapulmonary vascular compression and hypoxic vasoconstriction. With contrast-enhanced CT scanning, the impacted mucocele should remain of low attenuation and without enhancement, distinguishing it from vascular lesions such as arteriovenous malformation or centrally obstructing neoplasm. The lack of a prominent feeding vessel further excludes the diagnosis of interlobar sequestration. Allergic bronchopulmonary aspergillosis may have a similar “finger in glove” appearance; however, it lacks adjacent hyperlucency, is frequently hyperattenuating on non-enhanced CT scans, and is typically seen in patients with allergies, asthma, or cystic fibrosis.

Nuclear medicine imaging may be performed preoperatively to determine segmental contributions to pulmonary function. In these cases, marked hypoperfusion of the affected pulmonary parenchyma is frequently seen with absent or delayed ventilation. If ventilation through collateral channels does occur, there is typically delayed washout due to air trapping.

If neoplasm cannot entirely be excluded, fluorodeoxyglucose (FDG)-PET scanning most often demonstrates no focal increase in FDG uptake. Measured maximum standard uptake value should be less than that of the adjacent mediastinal blood pool. Few cases of increased FDG uptake have been reported, however, and they likely reflect simultaneous infection or local inflammation associated with mucostasis.

At MRI, high signal intensity of the mucocele on both T1- and T2-weighted sequences is seen in 86% of patients. These characteristics support the theory that mucoceles are partially fluid-filled irrespective of protein content. Although this information may be diagnostically useful in ambiguous cases, MRI rarely adds any new significant information not already afforded by routine chest CT scanning.

Pathologic Discussion

Gross surgical specimens reveal a soft, pliable mass representing the impacted mucocele. Involved segmental or lobar parenchyma may appear markedly overinflated and noncollapsible, with comparatively minimal anthracosis from relative anatomic isolation. Dissection fails to demonstrate a patent lumen between the distal and proximal bronchial tree; however, a remnant cord, bronchial septum, or bronchial membrane may be observed. Microscopically, alveoli appear hyperinflated but without destructive change, contrasting the bronchial atresia with congenital or acquired emphysema. Absolute and relative alveoli number decrease per unit volume of affected lung, most likely due to reduced ventilation, perfusion, and workload.

In the absence of superimposed infection, respiratory epithelium may exhibit focal thinning and absent or diminished pneumocytes with no signs of disruption or necrosis. Ciliated epithelium lines the impacted airways surrounding the mucocele, with functioning goblet cells scattered throughout. Histologic features of the remaining distal bronchial tree are typically normal.

Due to recurrent respiratory infections and persistent atypical chest pain, the patient was referred to cardiothoracic surgery for posterior segmentectomy of the right upper lobe. Postoperatively, she reported complete symptomatic resolution with significant improvement in air trapping at follow-up imaging. At 12 months, she continued to report no respiratory complaints and had recently competed in the New York City marathon.

Financial/nonfinancial disclosures: None declared.

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

Ramsay B.H. . Mucocele of the lung due to congenital obstruction of a segmental bronchus: a case report—relationship to congenital cystic disease of the lung and to congenital bronchiectasis. Dis Chest. 1953;24:96-103 [PubMed]journal. [CrossRef] [PubMed]
 
Lacquet L.K. .Fronhoff M. .Dierckx R. .Buyssens N. . Bronchial atresia with corresponding segmental pulmonary emphysema. Thorax. 1971;26:68-73 [PubMed]journal. [CrossRef] [PubMed]
 
Morikawa N. .Kuroda T. .Honna T. .et al Congenital bronchial atresia in infants and children. J Pediatr Surg. 2005;40:1822-1826 [PubMed]journal. [CrossRef] [PubMed]
 
Desir A. .Ghaye B. . Congenital abnormalities of intrathoracic airways. Radiol Clin North Am. 2009;47:203-225 [PubMed]journal. [CrossRef] [PubMed]
 
Kinsella D. .Sissons G. .Williams M.P. . The radiological imaging of bronchial atresia. BJR. 1992;65:681-685 [PubMed]journal. [CrossRef] [PubMed]
 
Jederlenic P.J. .Sicilian L.S. .Baigelman W. .Gaensler E.A. . Congenital bronchial atresia. A report of 4 cases and a review of the literature. Medicine (Baltimore). 1986;65:73-83 [PubMed]journal. [CrossRef] [PubMed]
 
Demos N.J. .Teresi A. . Congenital lung malformations: a unified concept and a case report. J Thorac Cardiovasc Surg. 1975;70:260-264 [PubMed]journal. [PubMed]
 
Kunisaki S.M. .Fauza D.O. .Nemes L.P. .et al Bronchial atresia: the hidden pathology within a spectrum of prenatally diagnosed lung masses. J Pediatr Surg. 2006;41:61-65 [PubMed]journal. [CrossRef] [PubMed]
 
Riedlinger W.F. .Vargas S.O. .Jennings R.W. .et al Bronchial atresia is common to extralobar sequestration, intralobar sequestration, congenital cystic adenomatoid malformation, and lobar emphysema. Pediatr Dev Pathol. 2006;9:361-373 [PubMed]journal. [CrossRef] [PubMed]
 
Gipson M.G. .Cummings K.W. .Hurth K.M. . Bronchial atresia. Radiographics. 2009;29:1531-1535 [PubMed]journal. [CrossRef] [PubMed]
 
Biyyam D.R. .Chapman T. .Ferguson M.R. .Deutsch G. .Dighe M.K. . Congenital lung abnormalities: embryologic features, prenatal diagnosis, and postnatal radiologic-pathologic correlation. Radiographics. 2010;30:1721-1738 [PubMed]journal. [CrossRef] [PubMed]
 
Pugatch R.D. .Gale M.E. . Obscure pulmonary masses: bronchial impaction revealed by CT. AJR. 1983;141:909-914 [PubMed]journal. [CrossRef] [PubMed]
 
Gohil H. .Muthukishnan A. . Bronchial atresia on FDG PET/CT: imaging characteristics of a rare pulmonary pseudonodule. Clin Nucl Med. 2012;37:86-87 [PubMed]journal. [CrossRef] [PubMed]
 
Wesselius L.J. .Muhm J.R. .Tazelaar H.D. . Congenital bronchial atresia: a case report with radiographic and pathologic correlation. Southwest J Pulm Crit Care. 2011;3:64-69 [PubMed]journal
 
Matsushima H. .Takayanagi N. .Satoh M. .et al Congenital bronchial atresia: radiologic findings in nine patients. J Comput Assist Tomogr. 2002;26:860-864 [PubMed]journal. [CrossRef] [PubMed]
 

Figures

Figure Jump LinkFigure 1 A and B, Posteroanterior and lateral radiographs of the chest demonstrates a tubular branching opacity overlying the region of the right upper lobe (white arrows) with adjacent paucity of pulmonary vascular markings.Grahic Jump Location
Figure Jump LinkFigure 2 A, Contrast-enhanced axial CT image of the chest with lung windows shows a tubular, nonenhancing lesion (white arrow) surrounded by an area of wedge-shaped hyperlucency (curved white arrows) within the right upper lobe. B, Oblique maximum intensity projection with lung-windows better emphasizes the tubular branching nature of the lesion (white arrow) as well as narrowed vasculature within affected parenchyma. C, Coronal minimum intensity projection with lung windows emphasizes wedge-shaped peripheral hyperlucency secondary to air trapping (white arrows).Grahic Jump Location
Figure Jump LinkFigure 3 A, Planar ventilation scintigraphy following inhalation of technetium-99m-labeled DTPA (diethylene-triamine-pentaacetate) aerosol shows a wedge-shaped ventilation defect radiating from the right hilum (white arrow). B, Fused single-photon emission CT/CT scanning following the injection of 4 mCi of technetium-99m-labeled macroaggregated albumin shows an associated perfusion defect (white arrow), corroborating CT findings of regional oligemia within the affected lung parenchyma.Grahic Jump Location
Figure Jump LinkFigure 4 A, Section from the resected mass at low-power magnification shows bronchiolar dilation with areas of mucostasis (arrows). B, Intermediate magnification reveals peribronchial fibrosis (curved arrow) with lymphocytic infiltrate (arrow). C, High-power magnification emphasizes diffusely dilated alveolar air spaces. Two lymph nodes sent separately for review were benign (not shown).Grahic Jump Location

Tables

References

Ramsay B.H. . Mucocele of the lung due to congenital obstruction of a segmental bronchus: a case report—relationship to congenital cystic disease of the lung and to congenital bronchiectasis. Dis Chest. 1953;24:96-103 [PubMed]journal. [CrossRef] [PubMed]
 
Lacquet L.K. .Fronhoff M. .Dierckx R. .Buyssens N. . Bronchial atresia with corresponding segmental pulmonary emphysema. Thorax. 1971;26:68-73 [PubMed]journal. [CrossRef] [PubMed]
 
Morikawa N. .Kuroda T. .Honna T. .et al Congenital bronchial atresia in infants and children. J Pediatr Surg. 2005;40:1822-1826 [PubMed]journal. [CrossRef] [PubMed]
 
Desir A. .Ghaye B. . Congenital abnormalities of intrathoracic airways. Radiol Clin North Am. 2009;47:203-225 [PubMed]journal. [CrossRef] [PubMed]
 
Kinsella D. .Sissons G. .Williams M.P. . The radiological imaging of bronchial atresia. BJR. 1992;65:681-685 [PubMed]journal. [CrossRef] [PubMed]
 
Jederlenic P.J. .Sicilian L.S. .Baigelman W. .Gaensler E.A. . Congenital bronchial atresia. A report of 4 cases and a review of the literature. Medicine (Baltimore). 1986;65:73-83 [PubMed]journal. [CrossRef] [PubMed]
 
Demos N.J. .Teresi A. . Congenital lung malformations: a unified concept and a case report. J Thorac Cardiovasc Surg. 1975;70:260-264 [PubMed]journal. [PubMed]
 
Kunisaki S.M. .Fauza D.O. .Nemes L.P. .et al Bronchial atresia: the hidden pathology within a spectrum of prenatally diagnosed lung masses. J Pediatr Surg. 2006;41:61-65 [PubMed]journal. [CrossRef] [PubMed]
 
Riedlinger W.F. .Vargas S.O. .Jennings R.W. .et al Bronchial atresia is common to extralobar sequestration, intralobar sequestration, congenital cystic adenomatoid malformation, and lobar emphysema. Pediatr Dev Pathol. 2006;9:361-373 [PubMed]journal. [CrossRef] [PubMed]
 
Gipson M.G. .Cummings K.W. .Hurth K.M. . Bronchial atresia. Radiographics. 2009;29:1531-1535 [PubMed]journal. [CrossRef] [PubMed]
 
Biyyam D.R. .Chapman T. .Ferguson M.R. .Deutsch G. .Dighe M.K. . Congenital lung abnormalities: embryologic features, prenatal diagnosis, and postnatal radiologic-pathologic correlation. Radiographics. 2010;30:1721-1738 [PubMed]journal. [CrossRef] [PubMed]
 
Pugatch R.D. .Gale M.E. . Obscure pulmonary masses: bronchial impaction revealed by CT. AJR. 1983;141:909-914 [PubMed]journal. [CrossRef] [PubMed]
 
Gohil H. .Muthukishnan A. . Bronchial atresia on FDG PET/CT: imaging characteristics of a rare pulmonary pseudonodule. Clin Nucl Med. 2012;37:86-87 [PubMed]journal. [CrossRef] [PubMed]
 
Wesselius L.J. .Muhm J.R. .Tazelaar H.D. . Congenital bronchial atresia: a case report with radiographic and pathologic correlation. Southwest J Pulm Crit Care. 2011;3:64-69 [PubMed]journal
 
Matsushima H. .Takayanagi N. .Satoh M. .et al Congenital bronchial atresia: radiologic findings in nine patients. J Comput Assist Tomogr. 2002;26:860-864 [PubMed]journal. [CrossRef] [PubMed]
 
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