Williams-Campbell syndrome is a rare form of congenital bronchiectasis, characterized by the absence, or marked diminishing of the cartilage surrounding the second or third through the sixth generation bronchi. The trachea, main and proximal bronchi are able to maintain their structural integrity. However, any respiratory infection and the subsequent small airway obstruction is capable of leading to bronchial collapse in these patients. Major symptoms seen in patients are intermittent cases of persistent cough and wheeze, pneumonia and broncho-obstructive symptoms, starting from around 3-years of age.
Earlier, diagnosis was made on the basis of bronchography, and fluoroscopic visualization. However, of late, multislice spiral dymanic CT has been shown to have exceptional significance in diagnosing this condition. Ballooning of the bronchi on inspiration and collapse on expiration, as seen on the CT scan are typical findings of the symptom. Prognosis is variable, with rapid clinical deterioration in some patients, and long-term survival in some others.