Williams-Campbell syndrome

Definition

A rare, respiratory malformation characterized by defective or completely absent bronchial wall cartilage in subsegmental bronchi, leading to distal airway collapse and contributing to the formation of bronchiectasis. The defect is mostly present between the fourth and sixth order bronchial divisions. Clinical manifestation includes recurrent pneumonia, coughing and wheezing.

Disease data

Classification

Morphological anomaly

ORPHA code

411501

OMIM code

ICD10 code

Q33.4

ICD11 code

*Soruce