Plastic Bronchitis Related to Idiopathic Thoracic Lymphangiectasia: Noncontrast Magnetic Resonance Lymphography

摘要

A 27-year-old woman presented with a 2-month history of sudden cough with thick sputum sometimes forming a cast of the bronchial tree (Figure 1A). She denied any medical or surgical history. She had a child via a normal vaginal delivery 6 months before. Physical examination was normal. Chest radiograph, cardiac ultrasound examination, and biological tests (including search for Aspergillus sensitization) were unremarkable. Sputum examination revealed numerous small lymphocytes. Tests for bacteria and fungi were negative. A computed tomography scan of the lung showed ground-glass opacities in the left upper lobe (Figure 1B), with septal thickening in the lower lobes. Noncontrast magnetic resonance (MR) lymphography (Figures 1C and 1D) showed dilated peribronchial pulmonary lymphatics and a dilated lymphatic network in the upper mediastinum. No abnormality was observed in the abdomen or pelvis. Bronchial biopsies revealed dilated lymphatic ducts (Figure 2). Plastic bronchitis resolved after strict median chain triglyceride diet but recurred after spontaneous discontinuation of the diet, which was poorly tolerated by the patient. Prompt and complete relief was obtained after reintroduction of the diet.