Ehlers-Danlos Syndrome Type IVB and Tracheobronchomegaly. Issue 1 (January 2018)

• Record Type: Journal Article
• Authors: Girit, Saniye; Senol, Ebru; Cag, Yakup; Karatas, Ozge; Baysal, Tamer
• Is Part Of: Journal of bronchology & interventional pulmonology. Volume 25:Issue 1(2018)
• Publication Date: 2018-01
• Subjects: Ehlers-Danlos syndrome -- hemoptysis -- Mounier-Kuhn syndrome -- tracheobronchomegaly; Bronchoscopy -- Periodicals; Lungs -- Diseases -- Periodicals; Respiratory Tract Diseases -- Periodicals; Lung Diseases -- surgery -- Periodicals; Diagnostic Techniques, Respiratory System -- Periodicals; Bronchi; Bronchoscopy; Lungs -- Diseases; Periodicals; 616.23
• DOI: 10.1097/LBR.0000000000000416
• Languages: English
• ISSNs: 1944-6586

Abstract

Abstract : Tracheobronchomegaly (TBM) is a rare disease of unknown etiology, characterized by dilatation of the trachea and main bronchi. Despite its rarity, TBM has been reported to accompany several conditions, particularly Ehlers-Danlos syndrome. Herein, we report on a 15-year-old patient who was diagnosed with TBM on the basis of radiologic and endoscopic findings and with Ehlers-Danlos syndrome type IVB presenting with marfanoid features, severe kyphoscoliosis, increased skin elasticity, and ongoing hypotonia since the neonatal period.