Faculty of Medicine and Health Sciences, Department of Paediatrics and Child Heath, Stellenbosch University and Tygerberg Hospital, Cape Town, South Africa.
Faculty of Medicine and Health Sciences, Division of Cardiothoracic Surgery, Stellenbosch University and Tygerberg Hospital, Cape Town, South Africa.
Pediatr Pulmonol. 2018 Jun;53(6):E18-E20. doi: 10.1002/ppul.24012. Epub 2018 Apr 10.
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.
纤维性纵隔炎（FM）是一种罕见的良性疾病，源于纵隔内过度纤维炎症反应，可压迫和阻塞纵隔结构。FM 有肉芽肿性和弥漫性非肉芽肿性两种形式。我们报告了一例可能由结核病引起的儿童肉芽肿性 FM 病例，该患儿气道、肺动脉和上腔静脉（SVC）严重受压，对治疗无反应，最终导致死亡。支气管镜检查发现气管支气管气道呈同心性狭窄和黏膜充血。该病例引起了对这种罕见并发症和有限治疗选择的认识。
