Plastic bronchitis (PB) is a pathologic condition in which airway casts develop in the tracheobronchial tree causing airway obstruction. There is no standard treatment strategy for this uncommon condition. We report an index patient treated using an emerging multimodal strategy of directly instilled and inhaled tissue plasminogen activator (t-PA) as well as 13 other cases of PB at our institution between 2000 and 2012. The majority of cases ( ) occurred in patients with congenital heart disease. Clinical presentations, treatments used, histopathology of the casts, and patient outcomes are reviewed. Further discussion is focused on the epidemiology of plastic bronchitis and a systematic approach to the histologic classification of casts. Comorbid conditions identified in this study included congenital heart disease (8), pneumonia (3), and asthma (2). Our institutional prevalence rate was 6.8 per 100,000 patients, and our case fatality rate was 7%. 1. Introduction: Index Case Plastic bronchitis, an uncommon condition of obstructive airway casts, has been reported in adults and children, predominantly in association with an underlying cardiac or pulmonary pathology. In order to illustrate the disease course and the array of therapeutic options, we present an index case. This case, which also illustrates a novel multimodal approach to plastic bronchitis treatment, is one of the 14 identified through a search of the electronic medical record at our institution over a 12-year period. We report a systematic comparison of cast histology as well as calculation of plastic bronchitis prevalence and mortality. The index patient, a 3-year-old male at the time of diagnosis with plastic bronchitis, was diagnosed with hypoplastic left heart syndrome by fetal echocardiography. Within hours of birth, he developed severe hypoxemia and underwent subsequent cardiac procedures including modified Norwood with placement of a right ventricle-to-pulmonary artery conduit, modified Blalock-Taussig shunt, bidirectional Glenn shunt, and extracardiac nonfenestrated Fontan. Pre-Fontan cardiac catheterization at 27 months of age showed normal pulmonary vascular resistance and an unobstructed Glenn circuit. After Fontan surgery, the patient developed hypoxia and low cardiac output from presumed intraoperative right lung injury. He required venoarterial extracorporeal membrane oxygenation (ECMO) for 5 days. The patient initially improved off of ECMO with normal Fontan pressures, but in the subsequent weeks his respiratory status declined. He developed increasing cough and 3 weeks
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