Congenital cystic bronchiectasis

Last revised by Andrew Murphy on 27 Jul 2022

Citation, DOI, disclosures and article data

Citation:

Gaillard F, Murphy A, Weerakkody Y, Congenital cystic bronchiectasis. Reference article, Radiopaedia.org (Accessed on 19 Apr 2024) https://doi.org/10.53347/rID-8865

DOI:

https://doi.org/10.53347/rID-8865

Permalink:

https://radiopaedia.org/articles/8865

rID:

8865

Article created:

5 Mar 2010, Frank Gaillard ◉ ◈

Disclosures:

At the time the article was created Frank Gaillard had no recorded disclosures.

View Frank Gaillard's current disclosures

Last revised:

27 Jul 2022, Andrew Murphy ◉

Disclosures:

At the time the article was last revised Andrew Murphy had no financial relationships to ineligible companies to disclose.

View Andrew Murphy's current disclosures

Revisions:

2 times, by 2 contributors - see full revision history and disclosures

Systems:

Chest, Paediatrics

Tags:

lungs

Congenital cystic bronchiectasis is a rare entity, and as the name suggests is characterized by cystic bronchiectasis found in infancy ¹. The term appears to have fallen out of favor, 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.