Congenital cystic bronchiectasis

A.Prof Frank Gaillard et al.

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.

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Article information

rID: 8865
Systems: Chest, Paediatrics
Tag: lungs
Synonyms or Alternate Spellings:

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