The role of spiral multidetector dynamic CT in the study of williams-campbell syndrome

Abstract

Williams-Campbell syndrome is a cystic bronchiectatic disease secondary to deficiency or defect of cartilaginous plates in the wall of the airways. In the literature, two main forms are suggested: congenital and acquired (post-infectious). The most frequent symptoms are represented by recurrent pulmonary infections from childhood. Multislice spiral dynamic CT has a major role in the study of cystic pulmonary disease and in differentiating Williams-Campbell syndrome from the other causes of cystic bronchiectasis, in which even lung function tests can give deceptive results.

Airway Malformations and Bronchiectasis: A Pediatric Study

“…In the characterization of WCS, spiral dynamic computerized tomography (CT) can assist in establishing the diagnosis of bronchiectasis. 7 The congenital malformation of the airway in WCS involves absence or diminished cartilaginous rings around the bronchi resulting in dilation and distal collapse. There has been at least 1 report of the familial occurrence of WCS among 2 related infants within 1 year of life.…”

Williams‐Campbell syndrome complicated with pulmonary hypertension and Type 2 respiratory failure: An adult case report

“…Pathology of the affected bronchi showing the deficiency of cartilaginous rings in the bronchial wall is the confirmatory test, but lung biopsy was not suitable for all the patients because of its complications. Di Scioscio et al described a case of WCS using inspiratory and expiratory CT imaging. On the same year, George et al vividly depicted the absence of the cartilage ring impressions in the bronchial wall, which were constructed from a volume of data acquired by thin‐slice CT scanning.…”

Williams–Campbell syndrome: a case report

“…The symptoms and prognosis depend on the extent of cartilage maldevelopment. CT imaging demonstrates bilateral cylindrical/cystic bronchiectasis distal to the third-generation bronchi (segmental/subsegmental) with hyperinflation of the lung 4,7. On expiration, collapse of the bronchi with distal air trapping is observed as the result of an excessively compliant bronchial wall.…”