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Thoracic Air Leakage in the Presentation of Cast Bronchitis FREE TO VIEW

Jonah Kruger, MD; Chaim Shpringer, MD; Elie Picard, MD; Eitan Kerem, MD
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From the Department of Pediatrics (Drs. Kruger and Kerem), Hadassah-Hebrew University Medical Center, Mount Scopus, Israel; Pulmonology Institute (Dr. Shpringer), Hadassah-Hebrew University Medical Center, Ein Kerem, Israel; and the Department of Pediatric Pulmonology (Dr. Picard), Shaare Zedek Medical Center, Jerusalem, Israel.

Jonah Kruger, MD, Hadassah-Hebrew University Medical Center, Mount Scopus, Department of Pediatrics, PO Box 24035, Jerusalem 91240, Israel; e-mail: jonahkruger@hotmail.com


Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/site/misc/reprints.xhtml).


© 2009 American College of Chest Physicians


Chest. 2009;136(2):615-617. doi:10.1378/chest.08-0383
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Published online

Background:  Cast bronchitis is a rare but potentially fatal condition caused by the accumulation of mucus in the bronchial tree usually secondary to infection, inflammation, or vascular stasis. Air leakage is an uncommon presentation of cast bronchitis. We report on three children with cast bronchitis who presented with acute air leakage as the main clinical feature.

Methods:  All three patients underwent bronchoscopic evaluation that led to a diagnosis and removal of bronchial casts.

Results:  Pathologic examination of casts from all three patients showed a mucoid substance with eosinophilic infiltration. The outcome for all patients was favorable.

Conclusions:  Acute respiratory failure presenting with wheezing and thoracic air leakage refractory to standard asthma therapy should raise suspicion of cast bronchitis, and urgent bronchoscopy should be performed.

Figures in this Article

Cast bronchitis is caused by the accumulation of mucus in the bronchial tree and leads to the formation of a plug, or cast, that takes the shape of the bronchial tree and causes airway obstruction.13 Cast formation is associated mainly with respiratory and cyanotic heart disease, with inflammation playing a key role in the pathogenesis.14 Air leakage as a presenting sign of cast bronchitis rarely has been reported. This article describes three cases of cast bronchitis in which the patients presented with acute respiratory failure and thoracic air leakage; all cases were definitively resolved with bronchoscopy.

Case 1

A previously healthy 13-month-old boy with a 2-day history of cough and fever was referred to the emergency department because of worsening dyspnea. On physical examination, the patient was in moderate respiratory distress, presenting with dyspnea with grunting, nasal flaring, and accessory muscle use. Subcutaneous emphysema was noted, and on auscultation, decreased air entry to the left lung was observed. A chest radiograph (Fig 1) showed a large pneumomediastinum, subcutaneous emphysema, and an infiltrate in the left upper lobe. Therapy with oxygen and salbutamol inhalations was ineffective. Approximately 24 h after hospital admission, bronchoscopy was performed during which a large plaque obstructing the main bronchus and branching into the right and left main bronchi was removed (Fig 2). Pathologic examination of the plaque revealed a mucoid substance with eosinophilic infiltration. Following the procedure, the patient's condition improved, the symptoms resolved, and he was discharged from the hospital receiving therapy with a bronchodilator and inhaled corticosteroids for 3 weeks. The results of sweat chloride testing for cystic fibrosis were negative. On a follow-up visit > 1 year later, the patient remained asymptomatic with no recurrence.

Figure Jump LinkFigure 1 Anterior-posterior chest radiograph showing pneumomediastinum, subcutaneous emphysema, and an infiltrate in the left upper lobe.Grahic Jump Location
Figure Jump LinkFigure 2 Cast extracted by rigid bronchoscopy.Grahic Jump Location
Case 2

A previously healthy 18-month-old girl presented to the emergency department with worsening dyspnea. She had been receiving amoxicillin therapy for suspected pneumonia. Prominent findings of the physical examination included marked respiratory distress with absent breath sounds over the left lung field. A chest radiograph showed collapse of the left lung with pneumomediastinum (Fig 3).

Figure Jump LinkFigure 3 Anterior-posterior chest radiograph showing collapse of left lung with pneumomediastinum.Grahic Jump Location

A bronchoscopy was immediately performed, revealing a large cast obstructing the left main bronchus. After removal of the cast, the patient's condition improved. She received therapy with a bronchodilator for 4 days while in the hospital and was discharged without the need for medication. The results of sweat chloride testing for cystic fibrosis were negative. Pathologic examination of the plaque showed a mucoid substance with eosinophilic infiltration. Six months after hospital discharge, the patient was asymptomatic with no subsequent recurrence.

Case 3

A previously healthy 38-month-old boy presented to the emergency department with acute respiratory distress. He had been ill with a dry cough for 3 days prior to hospital admission. On hospital admission, the patient was tachypneic, dyspneic, and cyanotic. Respiratory examination revealed diffuse rales and bilateral dullness to percussion at both lung bases. The chest radiograph obtained on hospital admission showed hyperinflation and increased pulmonary markings in the right lower lobe and left upper lobe. Treatment with bronchodilators and IV steroids proved ineffective. On the day of hospital admission, the patient's condition worsened, and a repeat chest radiograph showed bilateral pneumothorax (Fig 4).

Figure Jump LinkFigure 4 Anterior-posterior chest radiograph showing bilateral pneumothorax.Grahic Jump Location

Following bilateral chest tube insertion, urgent bronchoscopy was performed, and mucous casts were extracted from the right and left bronchi. Pathologic examination revealed a mucoid substance with eosinophilic infiltration. Following the procedure, the patient's condition rapidly improved, and he was discharged from the hospital receiving therapy with a bronchodilator and inhaled corticosteroids for 3 weeks. The results of sweat chloride testing for cystic fibrosis were negative. At the 1-year follow-up, the patient remained asymptomatic.

Cast bronchitis has been associated13 with multiple and varied causes, mainly with cardiac, respiratory, and hematologic conditions. Cyanotic heart disease remains the most common associated condition,4 particularly in patients post-Fontan. Cast formation as part of the acute chest syndrome in sickle cell anemia has been described5 and rarely has been associated6 with α-thalassemia. Historically, various classification systems have been developed in an attempt to better understand the pathophysiology. Seear et al7 originally proposed a pathologic classification system that divided casts into one of two types. Type I, or inflammatory, casts comprise fibrin, eosinophils, and Charcot-Leyden crystals associated with allergic and inflammatory conditions. Type II, or noninflammatory, casts comprise mucin and are associated with changes in vessel hydrostatics (eg, cyanotic heart disease). Subsequent identification8 of inflammatory-type casts associated with cyanotic heart disease, however, proved this classification system to be inadequate. Brogan et al3 suggested a classification system based on clinical presentation, dividing casts into allergic and asthmatic, cardiac, or idiopathic etiologies. Most recently, Madsen et al2 attempted to combine both classification systems, focusing initially on clinical presentation and further categorizing the casts based on pathology if the etiology is unclear. Regardless of classification, inflammation appears to be an important driving force in the production of casts. Further studies aimed at identifying inflammatory markers involved in the process may increase our understanding of the pathophysiology and advance therapeutic options.

Various therapies for cast bronchitis have been reported in the literature,1,8 ranging from simple patient expectoration to bronchoscopic removal. Other therapeutic methods have focused on cast destruction or disruption of formation and include tissue plasminogen activator, urokinase, steroids, mucolytic agents, and anticoagulants. The small number of cases of cast bronchitis has prevented the conducting of randomized trials, so the evidence remains anecdotal. The clinical signs of the condition are usually nonspecific and include dyspnea, wheezing, fever, and cough. Initial presentation can mimic status asthmaticus or foreign-body aspiration. The classically reported finding, bruit de drapeau (the sound of a flag snapping), thought to be caused by the sound of the mucus plug striking the airway wall, is heard only rarely; therefore, its relevance seems questionable. Radiographic signs include collapse of the involved segment and compensatory hyperinflation. More specific radiographic signs have been described, including presence of an elongated opacity with undulating borders in profile.1 A review of the literature3 shows that air leakage as a presenting sign of cast bronchitis rarely has been reported, with 4 of 42 cases described having presented with air leakage. Pneumomediastinum is particularly rare, having only been described in two previous cases.2 The mechanism of air leakage is likely due to the compensatory hyperinflation in an obstructed lung, leading to overdistention and rupture of lung parenchyma. Given the uncertain efficacy of various therapeutic options, a patient presenting with acute air leakage with suspected cast bronchitis should undergo prompt bronchoscopy.

The true incidence of cast bronchitis is unknown and may be significantly higher than reported, as most patients are treated symptomatically without undergoing bronchoscopy. In cases of wheezing with air leakage, refractory to standard asthma therapy and without clinical suspicion of foreign-body aspiration, physicians should consider cast bronchitis in making the differential diagnosis. Urgent bronchoscopy, therefore, is recommended, as the procedure is both diagnostic and the most reliable current therapeutic intervention.

Dr. Kruger gathered and reviewed the cases, wrote the initial article, rewrote the article after reviews by other authors, modified it according to CHEST reviewers' comments, and did the final review/edit of the last version of the article. Dr. Kerem was the main reviewer of the article. Dr. Shpringer and Dr. Picard were directly involved with the cases and were secondary reviewers of the articles as well.

The authors have reported to the ACCP that no significant conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

Noizet O, Leclerc F, Leteurtre S, et al. Plastic bronchitis mimicking foreign body aspiration that needs a specific diagnostic procedure. Intensive Care Med. 2003;29:329-331. [PubMed]
 
Madsen P, Shah S, Rubin B. Plastic bronchitis: new insights and a classification scheme. Paediatr Respir Rev. 2005;6:292-300. [PubMed] [CrossRef]
 
Brogan T, Finn L, Pyskaty J, et al. Plastic bronchitis in children: a case series and review of the medical literature. Pediatr Pulmonol. 2002;34:482-487. [PubMed]
 
Zaccagni HJ, Kirchner L, Brownlee J. A case of plastic bronchitis presenting 9 years after Fontan. Pediatr Cardiol. 2008;29:157-159. [PubMed]
 
Moser C, Nussbaum E, Cooper D. Plastic bronchitis and the role of bronchoscopy in the acute chest syndrome of sickle cell disease. Chest. 2001;120:608-613. [PubMed]
 
Vijayasekaran D, Sambandam AP, Gowrishankar NC. Acute plastic bronchitis. Indian Pediatr. 2004;41:1257-1259. [PubMed]
 
Seear M, Hui H, Magee F, et al. Bronchial casts in children: a proposed classification based on nine cases and a review of the literature. Am J Respir Crit Care. 1997;155:364-370
 
Nayar S, Parmar R, Kulkarni S, et al. Treatment of plastic bronchitis. Ann Thorac Surg. 2007;83:1884-1886. [PubMed]
 

Figures

Figure Jump LinkFigure 1 Anterior-posterior chest radiograph showing pneumomediastinum, subcutaneous emphysema, and an infiltrate in the left upper lobe.Grahic Jump Location
Figure Jump LinkFigure 2 Cast extracted by rigid bronchoscopy.Grahic Jump Location
Figure Jump LinkFigure 3 Anterior-posterior chest radiograph showing collapse of left lung with pneumomediastinum.Grahic Jump Location
Figure Jump LinkFigure 4 Anterior-posterior chest radiograph showing bilateral pneumothorax.Grahic Jump Location

Tables

References

Noizet O, Leclerc F, Leteurtre S, et al. Plastic bronchitis mimicking foreign body aspiration that needs a specific diagnostic procedure. Intensive Care Med. 2003;29:329-331. [PubMed]
 
Madsen P, Shah S, Rubin B. Plastic bronchitis: new insights and a classification scheme. Paediatr Respir Rev. 2005;6:292-300. [PubMed] [CrossRef]
 
Brogan T, Finn L, Pyskaty J, et al. Plastic bronchitis in children: a case series and review of the medical literature. Pediatr Pulmonol. 2002;34:482-487. [PubMed]
 
Zaccagni HJ, Kirchner L, Brownlee J. A case of plastic bronchitis presenting 9 years after Fontan. Pediatr Cardiol. 2008;29:157-159. [PubMed]
 
Moser C, Nussbaum E, Cooper D. Plastic bronchitis and the role of bronchoscopy in the acute chest syndrome of sickle cell disease. Chest. 2001;120:608-613. [PubMed]
 
Vijayasekaran D, Sambandam AP, Gowrishankar NC. Acute plastic bronchitis. Indian Pediatr. 2004;41:1257-1259. [PubMed]
 
Seear M, Hui H, Magee F, et al. Bronchial casts in children: a proposed classification based on nine cases and a review of the literature. Am J Respir Crit Care. 1997;155:364-370
 
Nayar S, Parmar R, Kulkarni S, et al. Treatment of plastic bronchitis. Ann Thorac Surg. 2007;83:1884-1886. [PubMed]
 
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