Congenital tracheomalacia refers to a diverse group of conditions which result in a dilated trachea with increased compliance. Causes include:
- cystic fibrosis: seen in up to 69% of patients 4
- Mounier-Kuhn syndrome: idiopathic or associated with
- Marfan syndrome
- Kenny-Caffey syndrome
- Cornelia de Lange syndrome
- Bruton-type agammaglobulinemia
Although all of these conditions are congenital, tracheomalacia may not become apparent until later in life, e.g. Mounier-Kuhn syndrome.
For a discussion of acquired tracheomalacia, and the general radiographic findings of tracheomalacia, please refer to tracheomalacia.
- 1. Hasegawa I, Boiselle PM, Raptopoulos V et-al. Tracheomalacia incidentally detected on CT pulmonary angiography of patients with suspected pulmonary embolism. AJR Am J Roentgenol. 2003;181 (6): 1505-9. AJR Am J Roentgenol (full text) [pubmed citation]
- 2. Webb EM, Elicker BM, Webb WR. Using CT to diagnose nonneoplastic tracheal abnormalities: appearance of the tracheal wall. AJR Am J Roentgenol. 2000;174 (5): 1315-21. AJR Am J Roentgenol (full text) [pubmed citation]
- 3. Reeder and Felson's gamuts in radiology. Maurice M. Reeder; with MRI gamuts by William G. Bradley, Jr., and ultrasound gamuts by Christopher R. Merritt and input from a distinguished 20-member subspecialty editorial board. New York : Springer, c2003. ISBN:0387955887 (find it at amazon.com)
- 4. McDermott S, Barry SC, Judge EE et-al. Tracheomalacia in adults with cystic fibrosis: determination of prevalence and severity with dynamic cine CT. Radiology. 2009;252 (2): 577-86. doi:10.1148/radiol.2522081956 [pubmed citation]