Plastic Bronchitis Following Fontan Procedure: A Case Report

Özet

Fibrinous bronchitis, also known as plastic bronchitis or pseudomembranous bronchitis, usually manifests clinically with non-productivecough and dyspnea. Prior to the expectoration of plaque formations, patients have a history of viral or other respiratory disease. Plasticbronchitis is an extremely rare and fatal complication associated with Fontan procedure. The condition is characterized by the formationof inspissated bronchial casts which may cause life-threatening airway obstructions. Although the pathogenesis of this condition remainsunclear, it is believed to involve elevated pulmonary venous pressure, increased central venous pressure, and endobronchial lymphatic leakage.A 9-year-old male patient with Down syndrome underwent Fontan procedure 29 months earlier due to complete endocardial cushion defectand single ventricular physiology. The patient presented to the emergency department due to sudden-onset respiratory distress and fever(38.5 °C). Bilateral diffuse sibilant rhonchi, secretory rales, and intercostal retractions were noted during pulmonary system examination.Posterior-anterior chest x-ray showed cardiomegaly (cardiothoracic ratio=0.6) and bilateral diffuse infiltration. While under treatment, thepatient experienced sudden-onset cough with expectoration of rubbery sputum in the form of branching bronchi-shaped casts. Samples ofthe inspissated sputum were sent to the Pathology and Microbiology departments for examination. The pathology report indicated fibrinoidmaterial composed of a small number of inflammatory cells and bacterial plaques. Normal bacterial flora was identified in microbiologicalculture. Clinical presentation can vary from mild clinical findings to life-threatening symptoms. As in our patient, the diagnosis is madeclinically, based on expectoration of bronchial casts or their detection during bronchoscopy. As our patient responded well to medicaltreatment, we proceeded with clinical follow-up. Plastic bronchitis is a very rare entity, and carries a poorer prognosis when it develops aftercongenital heart disease, as in our case.