We report the case of a 5-year-old boy who developed chronic plastic bronchitis after Fontan surgery for a complex congenital heart disease. During a new admission for acute exacerbation of plastic bronchitis, he started on a mucolytic treatment with inhaled rhDNAse instead of inhaled fibrinolytics because of the potential bleeding risk in a patient on combined coumarin and aspirin treatment. Respiratory symptoms resolved promptly, and the patient was discharged home on rhDNAse treatment. He remained clinically stable on rhDNAse treatment without further hospitalization until definitive treatment with dynamic lymphangiography and percutaneous embolization.
Keywords: Bronchial casts; Children; Congenital heart disease; Fontan physiology; Plastic bronchitis; Pulmonary lymphatics.