Williams–Campbell syndrome

Williams–Campbell syndrome (WCS) is a disease of the airways where cartilage in the bronchi is defective. It is a form of congenital cystic bronchiectasis. This leads to collapse of the airways and bronchiectasis. It acts as one of the differential to Allergic bronchopulmonary aspergillosis. WCS is a deficiency of the bronchial cartilage distally. Williams–Campbell syndrome (WCS) is a disease of the airways where cartilage in the bronchi is defective. It is a form of congenital cystic bronchiectasis. This leads to collapse of the airways and bronchiectasis. It acts as one of the differential to Allergic bronchopulmonary aspergillosis. WCS is a deficiency of the bronchial cartilage distally. It is thought to result from a deficiency of cartilage formation in the 4th to 6th order segmental bronchi. Most described cases present sporadically in early childhood. However, some have been diagnosed as late as adulthood. Due to its rarity it presents a difficulty in adult diagnoses, and its initial presentation can be confused with septic shock. Diagnosis requires an appropriate clinical history, the characteristic expiratory airway collapse on radiological investigation, and exclusion of other causes of congenital and acquired bronchiectasis. Pathology of the affected bronchi by bronchoscopy showing the deficiency of cartilaginous plates in the bronchial wall is the confirmatory test. However, lung biopsy has several complications and is not always diagnostic. Considering its non-invasive methodology, facility of execution, and good patient tolerance, multi-slice spiral CT or CT bronchoscopy should be the test of choice to study cystic lung diseases in particular WCS. Radiologically, the lungs are overinflated, and on bronchoscopy bronchomalacia is demonstrated. In 2011, Hacken et al. conducted a systematic review, aiming to answer what are the effects of treatments in people with bronchiectasis but without cystic fibrosis. There are not enough studies to prove if bronchopulmonary hygiene, physical therapy, mucolytics, inhaled hyperosmolar agents, oral corticosteroids, leukotriene receptor antagonists, short-acting beta 2 agonists, long-acting beta 2 agonists, or anticholinergic therapy is beneficial. However, there are some promising results in other approaches. This was previously attempted in a patient with end-stage lung disease secondary to WCS. Although the patient did not have proximal airway collapse prior to transplantation, his posttransplant course was complicated by the development of bronchomalacia of the right and left mainstem bronchi. The patient experienced recurrent pulmonary infections and died of bacterial pneumonia one year after transplantation. In 2012, the first WCS patient lung transplant with prolonged survival (approaching 10 years) was reported.