Lethal fibrosing mediastinitis in a child possibly due to Mycobacterium tuberculosis.
Savvas AndronikouRobert P GieJacque JansonPublished in: Pediatric pulmonology (2018)
Fibrosing mediastinitis (FM) is a rare benign disease resulting from an excessive fibro-inflammatory reaction in the mediastinum that can compress and occlude mediastinal structures. There is a granulomatous and a diffuse non-granulomatous form of FM. We present a case of granulomatous FM following possible tuberculosis in a young child who presented with severe compression of the airways, pulmonary artery and the superior vena cava (SVC), unresponsive to treatment, resulting in death. Bronchoscopic findings included concentric narrowing and mucosal hyperaemia of the tracheobronchial airways. This case raises awareness of this rare complication and the limited treatment options available.
Keyphrases
- interstitial lung disease
- pulmonary artery
- mycobacterium tuberculosis
- vena cava
- systemic sclerosis
- coronary artery
- pulmonary hypertension
- rheumatoid arthritis
- pulmonary arterial hypertension
- cystic fibrosis
- idiopathic pulmonary fibrosis
- mental health
- pulmonary tuberculosis
- lymph node
- inferior vena cava
- early onset
- low grade
- weight gain
- combination therapy
- ultrasound guided
- mass spectrometry
- drug induced
- body mass index