Fibrin airway cast obstruction: Experience, classification, and treatment guideline from Denver

Abstract

Background and Objectives Plastic bronchitis (PB) is a condition characterized by the formation of thick airway casts leading to acute and often life‐threatening airway obstruction. PB occurs mainly in pediatric patients with congenital heart disease (CHO) who have undergone staged surgical palliation (Glenn, Fontan), but can also occur after chemical inhalation, H1N1, severe COVID‐19, sickle cell disease, severe asthma, and other diseases. Mortality risk from PB can be up to 40%–60%, and no treatment guideline exist. The objectives herein are to develop a standardized evaluation, classification, and treatment guideline for PB patients presenting with tracheobronchial casts, based on our experience with PB at the Children's Hospital of Colorado in Denver. Methods We describe 11 patients with CHO‐associated PB (post‐Fontan [n = 9], pre‐Fontan [n = 2]) who presented with their initial episodes. We utilized histopathological analysis of tracheobronchial casts to guide treatment in these patients, utilizing our hospital‐wide guideline document and classification system. Results We found that 100% of post‐Fontan PB patients had fibrinous airway casts, while pre‐Fontan PB casts were fibrinous only in one of two patients (50%). Utilizing histopathology as a guide to therapy, PB patients with fibrin airway casts were treated with airway‐delivered fibrinolytics and anticoagulants, as well as aggressive airway clearance and other supportive care measures. These therapies resulted in successful cast resolution and improved survival in post‐Fontan PB patients. Conclusion We have shown an improved outcome in PB patients whose treatment plan was based on Denver's PB classification schema and standardized treatment guideline based on tracheobronchial cast histopathology.

The First Definition of Pulmonary Component of Hypereosinophilic Syndrome: Bronchial Casts

“…), congenital heart disease (CHD), and acute chest syndrome associated with sickle cell disease. 10 , 11 The presented case had no history of recurrent wheezing or atopy and did not respond to the asthma treatment. All these disorders and diseases including asthma were excluded with a detailed examination of the patient.…”

“…Fibrinolytic agents and anticoagulants (tPA, unfractionated heparin, and urokinase), dornase alpha against cellular DNA and NAC as mucolytic can be administered by nebulization or intraoperatively through bronchoscope. 5 , 11 Systemic steroids to decrease secretions and anti-inflammatory activity and bronchodilators to assist in airway clearance and to reduce bronchospasm can be given. 5 , 12 Hypertonic saline with chest physiotherapy can be employed in airway clearance.…”

Plastic Bronchitis—A Serious Rare Complication Affecting Children Only after Fontan Procedure?

“…Recently, Liptzin et al [ 40 ] suggested a classification scheme based on the presence (Class II) or absence (Class I) of fibrin, which appeared to be helpful in treatment guiding. In the case of Class I PB, where there is an absence of fibrin, treatment should focus on mucolytics and other accessory measures based on cast pathology for bacteria and inflammatory cells.…”

Pediatric Pulmonology 2022 year in review: Rare and diffuse lung disease

“…Typically, PB occurs in congenital heart disease patients who have undergone surgical palliation. Liptzin et al 26 described 11 patients with PB due to congenital heart disease and proposed a novel classification scheme to describe composition of airway casts based on the presence of fibrin and inflammation. Treatment suggestions provided are based on cast characterization.…”