Williams-Campbell syndrome: a rare case of congenital bronchiectasis: a case report

Williams-Campbell syndrome (WCS) is a rare congenital syndrome that can lead to bronchiectasis. Although commonly found in pediatric age groups, it can also be seen in adults due to delayed diagnosis with respiratory symptoms of cough, sputum, and wheezing.

A 31-year-old male presented with an incidental finding of bilateral cystic lesions in the lung field for which high-resolution computed tomography of the chest was done, which showed bilateral multiple cystic lesions without any clinical findings except mild inspiratory wheeze.

Any suspected case of bronchiectasis must undergo imaging: chest radiograph or computed tomography scan, along with blood investigations, sputum tests, and, if needed, a biopsy to help establish a diagnosis. Diagnosis of WCS is made after exclusion of common causes, which can be cystic fibrosis, foreign body aspiration, tuberculosis, COPD, and congenital causes like bronchomalacia, Mounier-Kuhn syndrome, tracheoesophageal fistula, etc.

Chest physiotherapy, antibiotic therapy, vaccination, and pulmonary rehabilitation are primary modes of treatment. En bloc lung transplantation can be done in some cases.

Diagnosis is made by ruling out all other causes of bronchiectasis, but a dynamic CT scan confirms the diagnosis. Chest physiotherapy and antibiotics are the treatment of choice.