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Ehlers-Danlos Syndrome Type IVB and Tracheobronchomegaly
Saniye Girit1, Ebru Senol2, Yakup Cag2
1Departments of Pediatrics, Division of Pediatric Pulmonology.
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.
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