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Plastic bronchitis is an extremely rare and potentially fatal complication after Fontan operation. It is characterised by expectoration of long, branching bronchial casts and can manifest with recurrent life threatening airway obstruction. The pathogenesis of this condition is not entirely clear. Contributing roles of elevated pulmonary venous pressure, increased central venous pressure, and endobronchial lymphatic leakage have been proposed. Various treatment modalities including thoracic duct ligation and cardiac transplantation have been proposed.
A 3.5 year old boy was admitted with acute respiratory distress, four weeks following Fontan operation for hypoplastic left heart syndrome. On admission he expectorated three large pearly white bronchial casts (left panel) with immediate symptomatic improvement. There was no history of atopy, allergy, or asthma. Total and differential white cell count was normal. Chest radiograph showed bilateral perihilar air space shadowing, no focal lung lesions, normal heart size, and no pleural effusion. Bronchial casts were composed of proteinacious and mucoid material with a few lymphocytes. There was no evidence of bacterial viral or fungal infection, and sweat test was negative.
Treatment with inhaled salbutamol and steroids, nebulised N-acetylcysteine, and antibiotics had no beneficial effect. He needed two emergency readmissions with bronchoscopic removal of a cast. Cardiac catheter at this stage confirmed unobstructed Fontan pathways and raised central venous pressure (15 mm Hg) with an acceptable transpulmonary gradient (5 mm Hg). Percutaneous creation of a stent fenestration in the Fontan circuit resulted in full symptomatic recovery from plastic bronchitis. At three years follow up he has no recurrence of respiratory symptoms. He remains cyanosed due to a persistent right-to-left shunt via the stent fenestration.