SERIES: RARE LUNG DISEASES
Plastic bronchitis: new insights and a classification scheme

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Summary

Plastic bronchitis is a rare disease in which there is the formation of large gelatinous or rigid branching airway casts. The history of the disease is reviewed and a classification system is proposed based on our current understanding. This system classifies plastic bronchitis by clinical disease association and cast histology. A summary of the medical literature is included together with guidelines for treatment. The evidence for these treatment decisions is reviewed.

Section snippets

INTRODUCTION

Plastic bronchitis is a rare disease characterised by the formation of large gelatinous or rigid branching airway casts. These casts are large and more cohesive than those seen in ordinary mucus plugging.1 The casts can be spontaneously expectorated and occasionally patients will cough up large impressions of their tracheobronchial tree (Fig. 1).2, 3 Patients can require bronchoscopy for cast removal4 and death due to airway obstruction from a cast is not uncommon.

Plastic bronchitis has been

CLASSIFICATION TAXONOMY

Before the 1950s, infections were the dominant comorbidity associated with plastic bronchitis. With the advent of antibiotics, atopic conditions and congenital heart disease (CHD) are now the most commonly reported associated diseases.3, 6, 20, 21, 22 Patients with SCACS can have plastic bronchitis complicating their course and that this complication may be under recognised.23

In 1902 Bettmann proposed a classification scheme based on the anatomic presentation and the presence of underlying

Structural CHD and mucin predominant casts

Although there are a few reports of patients with mitral regurgitation or pericarditis4, 7, 20 with plastic bronchitis, by far the most commonly associated clinical condition is cyanotic CHD.7, 10, 11, 12, 14, 15, 16, 17, 18, 31, 32, 33, 34, 35, 36, 37, 38 In most case reports, plastic bronchitis occurs after patients undergo a vascular diversion of systemic blood flow into the pulmonary circulation as part of a staged operation to correct underlying CHD defects and many patients have required

NATURAL HISTORY IN RELATION TO THE UNDERLYING DISEASE

The natural history of plastic bronchitis depends on the associated disease and cast type. Patients with structural CHD are at the greatest risk of death, regardless of the cast type and all reported deaths in these patients were from complications of airway obstruction with central propagation of the casts. The mean reported mortality in CHD associated casts is 33% (14–50%).

Patients who have chylous casts without CHD tend to be older (26–70 years) and no deaths have been reported. In most

THERAPY

Treatment has historically included bronchodilators, inhaled and oral corticosteroids, mucolytics, airway clearance therapy and antibiotics. Other therapies used have included inhaled heparin,30 urokinase,16 tissue plasminogen activator (TPA),36 dornase alfa9 and oral macrolide antibiotics as mucoregulatory therapy.47 There is only anecdotal evidence that any of these therapies is beneficial and this evidence has usually been provided for individual patients only.

Treatment for children with

FUTURE DIRECTIONS

There are many unanswered questions regarding the aetiology of this extremely rare disease. In published reports, neither abnormalities of the mucous glands and lymphatics nor the presence or absence of a clot or vascular injury have been reported. Most reports have not investigated the casts for the presence of chyle. Finally, there are no animal models of this disease that would enable hypothesis testing.

Only recently have techniques become available for evaluating the biopolymer composition

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      Although plastic bronchitis is a rare complication of multiple different disease processes, patients with CHD are at highest risk, with a mean reported mortality of 33% (14%-50%). However, in the current era, mortality of this disease is low, with more than 90% resolution of symptoms after lymphatic interventions.45 The prevalence of plastic bronchitis in patients after single-ventricle palliation is at least 4%.46

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