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.
George et al. (2006) reported the use of CT or virtual bronchoscopy (three dimensional reconstruction of the bronchial tree by using data obtained by thin slice CT scanning) in diagnosing a patient with Williams-Campbell syndrome. This female patient presented to the hospital with a lower respiratory tract infection and had a history of gradually progressive dyspnea, and dry cough over the period of three years during which she was admitted eight times with infective exacerbations. Clinically, she was tachypnoic with oxygen saturation of 48% on room air, had finger clubbing, central cyanosis, left parasternal heave, and a loud pulmonary second heart sound, as well as tender hepatomegaly. Chest examination revealed hyperinflation and bilateral coarse crackles maximal over mid zones. Blood investigations showed neutrophilia, three-four fold elevated liver enzymes, and prolonged prothrombin time. Analysis of arterial blood gasses revealed severe type II respiratory failure. Chest X-ray showed hyperinflation and bilateral extensive cystic bronchiectasis. ECG revealed p-pulmonale, and right ventricular hypertrophy. Proximal bronchiectasis with extensive peripheral centrilobular emphysema was confirmed by a high resolution chest CT scan, and features of Williams-Campbell syndrome (ballooning of the affected bronchi on inspiration, and collapse on expiration) were also found. Tuberculosis, immunodeficiency, cystic fibrosis, Aspergillosis, and nasal cilliary dysfunction were excluded. Serum alpha1-antitrypsin was normal. Pulmonary function tests showed severe mixed ventilatory defect with the obstructive component being irreversible. Virtual bronchoscopic images confirmed the expiratory collapse of the affected bronchi, as well as the absence of ring impressions, suggesting cartilage deficiency, which extended bilaterally from the mainstream bronchi to the subsegmental level, characteristic of Williams-Campbell syndrome. The patient was initially managed by 75% oxygen via a CPAP to maintain oxygen saturation >92%, as well as empirical antibiotics therapy, which were changed to Gentamycin and Ceftazidime as her sputum grew Pseudomonas aeruginosa. Regular chest physiotherapy was commenced as well. The patient's condition was stabilized on long term domicilary oxygen therapy at 3L/min with no admissions over a year. The authors suggested that this patient was a candidate for en bloc bilateral lung transplantation, rather than a sequential one as the cartilage was deficient in the main stem bronchi.