Treatment of plastic bronchitis in acute chest syndrome of sickle cell disease with intratracheal rhDNase. (Case Report).

Plastic bronchitis, a condition associated with widespread mucous plugging of the tracheobronchial tree, is an increasingly recognised bronchoscopic finding in acute chest syndrome of sickle cell disease. Removal of casts by bronchoscopy is technically challenging. We describe a child with acute chest syndrome where bronchoscopic removal of extensive tracheobronchial plastic casts was facilitated by intratracheal rhDNase.

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Acute chest syndrome (ACS) in sickle cell disease, which is characterised by fever, respiratory distress, and new pulmonary infiltrates, is a common cause of hospitalisation and accounts for 25% of deaths in these patients. (1) The underlying pathophysiological mechanisms may include pulmonary infection, infarction, sequestration, oedema, and bone marrow or fat embolism. Recently a high prevalence (72%) of plastic bronchitis, a condition associated with widespread mucous plugging of the tracheobronchial tree, has been reported in patients with ACS following flexible bronchoscopy. (2) The role of physiotherapy, mucolytics, and fibreoptic bronchoscopy for removal of these "plastic" casts has not been established.

Nebulised recombinant human DNase (rhDNase) is effective in clearing thick respiratory secretions in patients with cystic fibrosis. Intratracheal rhDNase has also been used in the treatment of life threatening mucus plugging in asthma (3) and lung atelectasis in neonates. (4) Here we describe a ventilated child with ACS complicated by plastic bronchitis who had a significant and sustained …