Congenital cystic bronchiectasis
Congenital cystic bronchiectasis is a rare entity, and as the name suggests is characterised by cystic bronchiectasis found in infancy 1. The term appears to have fallen out of favour, due to variable use in older literature. In most instances of congenital cystic bronchiectasis has what is now referred to as Williams-Campbell syndrome, and is the result of a deficiency of cartilage in the bronchial wall. Some authors use the terms synonymously 5.
Some cases may have been manifestations of Mounier-Kuhn syndrome.
Additionally, the term was used erroneously in older publications to refer to congenital pulmonary airways malformations (CPAM) 4.
As such it is probably best to avoid the term.
- 1. Aliabadi P, Shafiepoor H. Bronchography in the recognition of congenital cystic bronchiectasis. AJR Am J Roentgenol. 1978;131 (2): 255-7. AJR Am J Roentgenol (abstract) [pubmed citation]
- 2. Marom EM, Goodman PC, McAdams HP. Diffuse abnormalities of the trachea and main bronchi. AJR Am J Roentgenol. 2001;176 (3): 713-7. AJR Am J Roentgenol (full text) [pubmed citation]
- 3. Williams HE, Landau LI, Phelan PD. Generalized bronchiectasis due to extensive deficiency of bronchial cartilage. Arch. Dis. Child. 1972;47 (253): 423-8. doi:10.1136/adc.47.253.423 [free text at pubmed] [pubmed citation]
- 4. Frederick Taylor Lord "Diseases of the bronchi, lungs, and pleura" 1915. Google Books
- 5. Thurlbeck's Pathology Of The Lung. Andrew M., M.D., Ph.D. Churg, Jeffrey L., M.D. Myers, Henry D., M.D. Tazelaar, William M. Thurlbeck (Editor), Andrew Churg (Editor). Thieme Medical Publishers ISBN:1588902889 (find it at amazon.com)