Abstract
Background: Lymphatic plastic bronchitis (PB) most commonly
occurs in children with congenital heart disease as a result of
secondary pulmonary lymphatic flow disorder (PLFD). However, PB caused
by primary PLFD is rare. We made a retrospective analysis of two
children diagnosed with PB due to primary PLFD, in order to contribute
to further understanding of these disorders. Results: Patient
1, an eight-year-old boy, presented with chronic productive cough and
expectorated milky-white mucous plugs accompanied by intermitted
wheezing for one year. Patient 2, a nine-month-old girl, presented with
episodes of acute respiratory distress with expectoration of milky-white
bronchial casts for four months. There was no obvious evidence of
infection in either child. Bronchoscopy showed massive milky-white casts
blocking the airway in patient 2; no casts were observed in patient 1.
Bilateral thickening of bronchovascular bundles and interlobular septal,
as well as multiple patchy ground-glass opacities were seen on chest
computed tomography (CT) in both patients. Lymphangioscintigraphy
demonstrated pulmonary lymph reflux in both patients and slowed
lymphatic drainage of the lower limbs in patient 1. Primary PLFD was
considered for both patients, and a diagnosis of yellow nail syndrome
was made in patient 1. Both patients received lymphatic interventional
treatment, but all experienced recurrence following the procedure.Conclusions: Primary PLFD is a rare but significant cause of PB
in children. Chest CT findings have highly suggestive significance for
the diagnosis. The lymphatic interventional procedure may be effective
for short-term resolution of symptoms, but prone to recurrence.