Management of plastic bronchitis with nebulized tissue plasm
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Plastic bronchitis (PB) is an uncommon condition characterized by mucoid impaction with casts that occlude major bronchi and cause acute, potentially fatal bronchial obstruction. PB has been associated with a variety of respiratory diseases, but is found most commonly as a secondary phenomenon after Fontan surgery in patients with cyanotic congenital heart disease (CHD). Especially in these patients, recurrences are common and mortality remains high. Patients either expectorate casts spontaneously, coughing up a firm mucus material, or may require urgent bronchoscopy to remove casts and avoid impending respiratory arrest due to airway obstruction.

The pathophysiology of PB remains to be elucidated. It has been suggested that high pulmonary venous pressures may lead to an abnormal response of respiratory epithelium and/or lymphatic dysfunction, thus resulting in excess mucus production and cast formation. An underlying genetic predisposition associated with an inflammatory trigger was also postulated to explain the abnormal mucin hypersecretion. PB has been classified in type 1 (inflammatory) and 2 (noninflammatory) according to the characteristics of casts. Because inflammation is thought to play a role in the formation of all casts, Madsen et al. have recommended classification based on associated disease first and on cast histology second.

We report the case of an adolescent with CHD and primary ciliary dyskinesia (PCD) who developed features of PB and severe respiratory failure 7 yrs apart from Fontan operation. The occurrence of PB in our patient let us hypothesize that decreased mucociliary clearance was an adjunctive trigger for the development of PB. The effect of early, intermittent nebulization of recombinant human tissue plasminogen activator (t-PA) therapy is described....