Plastic bronchitis is a rare fatal disease characterized by the airway obstruction due to bronchial casts. Though causes of plastic bronchitis remain unclear, some cases of plastic bronchitis have occurred after Fontan operation. We present a patient with plastic bronchitis associated with Fontan operation in whom correction of imbalanced pulmonary blood flow (PBF) is effective.
This patient was a 6-year-old boy with hypoplastic left heart syndrome. He had been treated a modified Norwood operation with RV-PA shunt at 7 days of age; a bidirectional Glenn operation at 6 months; a fenestrated, lateral tunnel Fontan operation at 2years. At the age of 6 years he was admitted to our hospital because of acute episode of dyspnea. During admission he coughed up a dendritic mucoid bronchial cast. This cast consisted of fibrin and mucin, and contained eosinophils as well as inflammatory cells. He was diagnosed as plastic bronchitis, and was treated with both inhaled and oral corticosteroid (CS). Though CS therapy was effective, plastic bronchitis often recurred shortly whenever CS therapy was reduced. Bronchoscopic examination during dyspnea episode revealed bronchial cast located in right bronchus. By cardiac catheter examination left pulmonary artery stenosis was confirmed, and lung perfusion scintigram showed relative high right PBF. We implanted a stent percutaneously for the left pulmonary artery stenosis, and then high right PBF decreased. After stenting, plastic bronchitis had no recurrence only use of a low dose CS therapy.
We suggest correction of imbalanced PBF may benefit patients in whom other therapies have failed.