Treatment of Plastic Bronchitis in a Fontan Patient With Tissue Plasminogen Activator: A Case Report and Review of the Literature

Abstract

ABSTRACT. Plastic bronchitis is a condition in which large, bronchial casts with rubber-like consistency develop in the tracheobronchial tree and cause airway obstruction. We describe a 4-year-old girl who had Fontan physiology and who developed plastic bronchitis and report for the first time the use of aerosolized tissue plasminogen activator for treatment of this condition. The literature is reviewed with emphasis placed on the occurrence of this disorder in patients with single ventricle physiology. Pediatrics 2002;109(4). URL: http:// www.pediatrics.org/cgi/content/full/109/4/e67; plastic bronchitis, bronchial cast, Fontan, congenital heart disease, tissue plasminogen activator.ABBREVIATION. t-PA, tissue plasminogen activator. P lastic bronchitis is a condition in which large, pale tan, bronchial casts with rubber-like consistency develop in the tracheobronchial tree and cause airway obstruction. 1 It is an unusual disorder that occurs in various disease states, and fewer than a dozen cases have been reported regarding patients with palliated congenital heart disease. We describe a 4-year-old girl who had Fontan physiology and who developed plastic bronchitis and report for the first time the use of aerosolized tissue plasminogen activator (t-PA) for treatment of this condition. The literature is reviewed with emphasis placed on the occurrence of this disorder in patients with single ventricle physiology. CASE REPORTOur patient's double-inlet left ventricle and L-transposition of the great arteries were diagnosed at birth. Her cardiac palliation performed at an outside institution consisted of a pulmonary artery band at 1 month of age; a bidirectional Glenn operation at 12 months; and a fenestrated, lateral tunnel Fontan operation at 26 months. Fenestration closure using a subcutaneous snare was performed 6 months later. Cardiac catheterization at 48 months of age revealed excellent hemodynamics without Fontan pathway obstruction. The mean pressure in the lateral tunnel was 10 mmHg, and there was no gradient to the left or right pulmonary arteries. The left ventricular end diastolic pressure was 4 mmHg, and the bulboventricular foramen was unobstructed.At 53 months of age, she presented to our institution with respiratory failure requiring mechanical ventilation. She improved with nebulized bronchodilator therapy and chest physiotherapy. This episode of respiratory decompensation was attributed to a mucus plug. During the next 4 months, she had 4 additional hospital admissions for perceived asthma exacerbations and pneumonia. Chest radiographs on each of these admissions revealed shifting infiltrates and atelectasis. A bronchoscopy performed during the third admission was unremarkable. The diagnosis of plastic bronchitis was made when the patient's parent brought an expectorated bronchial cast to the pediatrician after the fourth admission and bronchoscopy during a subsequent admission revealed a large bronchial cast. She was then treated on an outpatient basis with chest physiotherapy, inhaled steroids, al...

Plastic Bronchitis in a Post-Fontan Pediatric Patient With Normal Hemodynamics: A Case Report

“…Our case is unique in several aspects: a review of 15 patients who developed PB after undergoing Fontan surgery showed that a majority of postFontan PB had mucinous casts, but our patient's casts consisted of mucin, fibrin, and inflammatory cells, similar to those described by Costello et al 5 Secondly, our patient had normal extracardiac Fontan mean pressure of 11 mmHg, which is lower than the mean pressure of patients described in the literature .2,5 Our case did not have any of the risk factors as reported by Schumacher et al 2 that could have triggered an earlier therapeutic response.…”

“…al's 25 cases 2 of PB had an average post-Fontan PAP of 16.5 mmHg (range 12 to 22) compared to 43 non-PB Fontan patients with average PAP of 13 mmHg (range 12 to 15), but 39% of the non-FB patients did not have available PAP information. Costello et al 5 also reported a post-Fontan PB with lateral tunnel mean pressure of 10 mmHg, where the cast was found to consist of fibrin, mucin, and inflammatory cells. Tzifa et al 9 reported a case of postFontan PB where Fontan mean pressure was 13 mm Hg, the cast consisted of fibrin and neutrophils, and the cast culture grew Staphylococcus aureus.…”

Bronquite plástica em criança com talassemia alfa

“…There are, however, proposals that additional treatment be given, which could include corticosteroids (2 mg/kg/day, in the case of type I molds), 9 antibiotics 9 for associated bacterial conditions, broncho-alveolar lavage, respiratory physiotherapy and topical N-acetyl-cysteine (2 to 4 ml of 10% solution). [10][11][12] More recently, the use of thrombolytics in aerosol form (urokinase) and the endotracheal instillation of recombinant immunoglobulin (Dornase Alpha) have been described. 12 Mechanical removal of the molds under direct observation (bronchoscopy) can be very useful in cases of children with acute respiratory insufficiency.…”

Successful Management of Plastic Bronchitis in a Child Post Fontan: Case Report and Literature Review

“…Case studies have described the use of inhaled therapies [2,12,[53][54][55][56], anti-inflammatories [57,58], diuretics [38], and surgical palliation [34,35,59,60]. Brogan et al [2] incubated a cast from a Fontan patient in vitro with acetylcysteine, DNase, urokinase, and tPA, demonstrating that acetylcysteine had the greatest effect in dissolving the casts.…”

“…Quasney et al [56] 123 benefit for use of tPA in plastic bronchitis [36,61]. However, there has been reported effective use of inhaled tPA [55,62], and Wakeham et al [63] showed long-term and efficacious therapy over the course of 18 months without any adverse complications. A murine model studied the safety of prolonged, repeated administration of inhaled pulmonary formulation tPA [64].…”