Aggressive bronchoscopic management of plastic bronchitis.

Plastic bronchitis or "Bronchitis Plastica" is a rare disease characterized by the formation of thick, tenacious, arborizing mucofibrinous tracheobronchial casts that result in life-threatening airway obstruction and pulmonary failure. We review three children who developed recurrent plastic bronchitis after undergoing a Fontan procedure for single ventricle physiology. Case series of three patients with plastic bronchitis at a tertiary referral children's hospital. All patients required repeated bronchoscopies, one requiring four separate ones over a week's period, for removal of the rigid casts. Extra-corporal membrane oxygenation (ECMO) was needed in two children because of severe respiratory failure. All were also managed with adjunctive intensive medical support. Pulmonary function returned to normal in all children, but recurred 2 months later in one who subsequently expired due to pulmonary failure. Plastic bronchitis is an unusual condition of unknown cause that occurs in multiple clinical settings, but especially in those children who have undergone a Fontan operation. Management of this distressing situation is difficult and early diagnosis and aggressive measures to remove rigid casts combined with intensive medical care are necessary. The intrinsic cardiopulmonary physiology of children with Fontan procedures, including the risk of arrhythmias, hypo-oxygenation, and pulmonary hypertension make this condition even more complex.