Background
Plastic bronchitis (PB) is a rare disease characterized by the
expectoration of rubbery, cohesive, and branched bronchial casts that
obstruct the airway partially or totally. BP is associated with
respiratory disorders such as bronchopulmonary infection, asthma,
allergic bronchopulmonary aspergillosis (ABPA), and cystic fibrosis.
However, it most commonly occurs in children with congenital heart
disease, especially those with single ventricle physiology following the
Fontan procedure. Elevation of central venous pressure after the
procedure and secondary pulmonary lymphatic flow disorder (PLFD),
leading to abnormal perfusion of the bronchial submucosa with lymph and
formation of the airway cast 1-2. ‘Lymphatic PB’ is
used to differentiate it from PB related to other causes3. However, PB caused by primary PLFD is rare and
the clinical manifestation and
severity vary among patients; thus, the diagnosis can be challenging,
especially in the absence of chylothorax.
In this paper, we report two cases
of primary PLFD presenting primarily as PB, in order to contribute to
further understanding of these disorders.