Abstract
RF, 10 years old girl was first observed when she was 9 years and 8 months old, for suspected plastic bronchitis, after the release of a cast during an acute episode of asthma.
Clinical history was uneventful up to 2009, when she suffered from an acute pneumonia. From September 2011 she presented recurrent bronchial asthma.
In march 2012 she had a persistent bronchospasm with progressive worsening of general conditions and expectoration of a cast; she underwent Bronchoscopy, Chest CT, angioMRI, lymphoscintigraphy that showed a lymphobronchial fistula with dysplasia of lymphatic return and stasis in the lung. Bronchoalveolar lavage and aspiration liquid chemical analysis showed a high amount of tryglicerides and no-esterified fatty acids. A low fat diet with integration of MCT oil was started with initial clinical improvement. After one month she presented again respiratory symptoms worsening and chiloptisi.
Chest X ray demonstrated a wide interstitial lung disease.
A plasminogen activator (altoplase) inhaled treatment was started, with subsequent clinical improvement.
In October 2012 she had a new clinical worsening, she underwent a total parenteral nutrition.
In order to differentiate a primary from a secondary pulmonary lymphangectasis, we performed a lung biopsy that proved to be consistent with a secondary form.
She underwent ligation of the thoracic duct, to exclude the portion of the abdominal lymph tributary lung.
After two weeks she was discharged without pharmacological treatment and cholesterol-free diet.
After 40 days from discharge we have witnessed a partial recovery of lung function with normal daily activities recovery and after three months an almost complete recovery of lung function.
- © 2013 ERS