(congenital bronchomalacia)

Rare. Absence of cartilage in the wall of subsegmental bronchi, which causes their collapse during expiration and their expansion during inspiration. This quickly leads to the formation of bronchiectasis at the level of the proximal subsegmental bronchi. The typical Xray picture is that of bronchiectasis in the bronchi beyond the 3rd generation associated with zones of hyperinflation and expiratory collapse of the distal bronchi.

Symptoms: frequent respiratory infections, pseudo-asthma. Symptoms appear in childhood (a few familial forms have been described) but the less severe forms may only be diagnosed in adulthood.

Differential diagnosis: cystic fibrosis, aspergillosis, chronic immune deficiency.

Anesthetic implications:

pre- and postoperative respiratory physiotherapy. Pulmonary emphysema. Airway hyperreactivity.

References : 

-         Jones QC, Wathen CG. 
Williams-Campbell syndrome presenting in an adult. 
BMJ Case Reports 2012 doi 10.1136/bcr-2012-006775

-        Jones VF, Eid NS, Franco SM, Badgett JT, Buchino JJ. 
Familial congenital bronchomalacia: Williams-Campbell syndrome. 
Pediatr Pulmonol 1993; 16: 263-7.

-        Shankar G, Kothari P, Sarda D et al. 
Pneumonectomy in a child with congenital bronchiectasia : a case report and review of the literature. 
Ann Thor Med 2006; 1: 81-3.

Updated: August 2018