Abstract
Primary lymphangiectasia is a rare congenital (variable age of presentation) disorder of the lymphatic system characterized by pronounced enlargement of the lymphatic vessels resulting in their obstruction and subsequent leakage of lymph fluid to the perilymphatic tissues. We describe young female patient with intestinal, pleural, and pulmonary involvement.
Pulmonary involvement presented with bilateral hospital aquired pneumonia connected with fever and dyspnea. At repetitional bronchoscopies, performed because of worsening of the clinical status, several endobronchial obstructions were found due to branched tenacious mucoid impactions.
The biggest mucoid endoluminal obstructions(copying minimally 8 generations of bronchial ramifications) were repeatedly removed from right B6.