SERIES: RARE LUNG DISEASESPlastic bronchitis: new insights and a classification scheme
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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A case of plastic bronchitis with a remarkable response to steroids
2023, Respiratory Medicine Case ReportsPlastic bronchitis and Glenn-stage heart disease
2022, Anales de PediatriaLymphatic Disorders and Management in Patients With Congenital Heart Disease
2022, Annals of Thoracic SurgeryCitation Excerpt :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
Impact of preoperative left pulmonary artery stenting on the Fontan procedure: a retrospective multicentre study
2024, European Journal of Cardio-thoracic Surgery