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.
Contents
Symptoms
- Persistent cough
- Wheeze
- Impaired lung function
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.
History
It was described in 1960 by Howard Williams and Peter Campbell.
References
Williams–Campbell syndrome Wikipedia(Text) CC BY-SA