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Williams–Campbell syndrome

Abstract

Williams–Campbell syndrome also known as bronchomalacia 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. Williams–Campbell syndrome is deficiency of the bronchial cartilage distally.

Pathology

It is thought to result from a deficiency of cartilage formation in the 4th to 6th order segmental bronchi.

Diagnosis

Radiologically, the lungs are overinflated and on bronchoscopy bronchomalacia is demonstrated.