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

Abstract

Background: Plastic bronchitis (PB) may occur not only in children following palliative Fontan procedure but also in those without underlying heart disease. We aim to assess the clinical course, therapeutic measures, outcome, and follow-up of PB in children with congenital heart disease (CHD) and children without cardiac problems. Methods: This retrospective case series assessed children with PB admitted to hospital between 2015 and 2019. Parents or guardians of patients were contacted by e-mail or telephone between September 2017 and June 2019 to enquiry about recurrence of PB and strategy of treatment. The diagnosis of PB was based on the expectoration (spontaneous or during bronchoscopy) of endobronchial plugs. Results: This study delineated the clinical, histological, and laboratory features of plastic bronchitis in children following Fontan procedure (Group A) and in those without heart defects (Group B, non-CHD children). The main symptoms were cough accompanied by dyspnea, and hypoxemia with a decrease in oxygen saturation, often leading to acute respiratory failure. In children with CHD, the first episode of PB occurred at a relatively young age. Although chronic, i.e., lasting more than 3 weeks, inhaled therapy was implemented in both groups of patients, the recurrences of PB were observed. The mean time to PB recurrence after the first episode in Group A was longer than that in Group B (1.47 vs. 0.265 years, p = 0.2035). There was no re-episode with recurrence of PB in 3 cases out of 10 in total in Group A (30%) and 1 case out of 4 in total in Group B (25%). While the majority of children in Group A usually developed bronchial casts on the right side, the patients in Group B (without CHD) suffered from bronchial casts located only on the left side. Conclusions: Despite many similarities, clinical, histological, and laboratory studies in the children with plastic bronchitis after Fontan’s surgery and in children without heart defects suggest that there are differences in the course of the disease in patients without CHD, such as a more advanced age of the first episode of PB, the location of plastic casts on the left side, and a stronger role of inflammatory factors and mechanisms. Further research is needed to understand the pathophysiology of PB and choose the most appropriate therapy.

Clinical features of plastic bronchitis in children after congenital heart surgery

“…The present study found that the pathology of PB with right ventricular dysfunction showed a small number of neutrophils and fibrin infiltration, which was consistent with the pathology of type 2 PB [7]. Studies have shown that the occurrence and development of type 2 PB are related to abnormal lymphatic circulation (10)(11). Children after Fontan or Glenn surgery may be due to the high flow of pulmonary blood vessels or increased pulmonary vascular resistance, which leads to the increase of intrapulmonary vascular exudation and the aggravation of a load of intrapulmonary lymphatic circulation (12)(13).…”

Plastic bronchitis during childhood: Diversity of presentation, etiology, treatment, and outcomes

“…They reported that patients with CHD usually developed bronchial casts on the right side, and patients without CHD usually developed bronchial casts on the left side. 14 Lung function tests play an important role in evaluating respiratory diseases such as asthma; however, lung function data reported from patients with PB are limited. 11 A recent study showed that three patients with mild airflow obstruction had normal lung function tests during follow-up and had stopped ICS treatment.…”

“…Most patients in our study were diagnosed as having nonstructural heart disease, and they had similar cast localizations with cast development on the left side. Pałyga‐Bysiecka et al 14 compared the characteristics of children with CHD and children without CHD among patients with PB. They reported that patients with CHD usually developed bronchial casts on the right side, and patients without CHD usually developed bronchial casts on the left side 14 …”

“…Pałyga‐Bysiecka et al 14 compared the characteristics of children with CHD and children without CHD among patients with PB. They reported that patients with CHD usually developed bronchial casts on the right side, and patients without CHD usually developed bronchial casts on the left side 14 …”

Clinical features of plastic bronchitis in children after congenital heart surgery

“…Studies have shown that the occurrence and development of type 2 PB are related to abnormal lymphatic circulation (10)(11). Children after Fontan or Glenn surgery may be due to the high ow of pulmonary blood vessels or increased pulmonary vascular resistance, which leads to the increase of intrapulmonary vascular exudation and the aggravation of a load of intrapulmonary lymphatic circulation (12)(13).…”