SESSION TITLE: Bronchology Cases I
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Sunday, October 27, 2013 at 01:15 PM - 02:45 PM
INTRODUCTION: We present a patient with persistent cough, worsening shortness of breath and bilateral pulmonary infiltrates that was found to have large bronchial casts and pathology confirmed the rare diagnosis of Plastic Bronchitis.
CASE PRESENTATION: A 61 year old male with history of chronic bronchitis on home oxygen therapy had multiple hospital admissions for recurrent fever, productive cough, bad taste and worsening dyspnea. He received multiple courses of antibiotics although previously no pathogenic organisms were isolated. His physical examination showed oxygen saturation of 84% on room air and bilateral wheezing. The immunological and vasculitis workup was negative. His IgE was 31, aspergillus precipitins and antibodies were negative. A year old CT chest (figure 1) showed bilateral pulmonary infiltrates and on the latest chest imaging there were multiple ground glass opacities (figure 2). The pulmonary function tests showed a progressive decline. Patient had a bronchoscopy done showing multiple bronchial casts (figure 3 and 4), which were removed. The histological specimen (figure 5) showed fibrin, mucin and neutrophil predominant cellular material which was consistent with diagnosis of inflammatory form of plastic bronchitis. He required multiple therapeutic bronchoscopies and was treated with both inhaled and high dose systemic steroids.
DISCUSSION: Plastic bronchitis has been described mostly in children. There is expectoration of large bronchial casts, which are of more cohesive and rubbery consistency than ordinary mucus plugging. Ventilgeraeusch (sound of a fan) or Bruit de drapeau (sound of a flag snapping) is typically present if the casts are subtotally obstructing the airway. Radiologically either atelectasis or infiltrates can be seen. Type 1 inflammatory form is characterized by bronchial disease and inflammation with presence of fibrin, mucin and cellular material mostly eosinophils on histology. Whereas, type 2 acellular form is without inflammation and has mainly mucin with little fibrin and is mostly associated with cyanotic heart diseases1. Both forms have a high mortality. Our patient had type 1 inflammatory plastic bronchitis. This disease is a management challenge2 and the patients usually require frequent bronchoscopies to remove the casts. Macrolides3, steroids, acetylcysteine, rhDNAase and aerosolized tPA have been tried only based on anecdotal case reports.
CONCLUSIONS: Plastic Bronchitis which has a high mortality causes a wide range of pulmonary complaints. The patients usually require frequent therapeutic bronchoscopies to remove the bronchial casts. Although it has no definitive therapy, there is some success with anecdotal case reports based therapy.
Reference #1: Am J Respir Crit Care Med. 1997 Jan;155(1):364-70.
Reference #2: Am J Med Sci. 2008 Feb;335(2):163-9.
Reference #3: Pediatr Pulmonol. 2003 Feb;35(2):139-43.
DISCLOSURE: The following authors have nothing to disclose: Syed Ali Riaz, Priyank Desai, Brian Fouty
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