William Campbell Syndrome as a Cause of Asymmetrical Bronchiectasis In a 10-Year-Old Child: A Case Report

Abstract

Background: William Campbell Syndrome is a rare congenital disease of the bronchial cartilages involving the 4th to 6th generation of bronchial divisions with involvement of bilateral lungs. There is deficiency of bronchial cartilages resulting in dilated bronchi, with collapse of the distal lung. Diagnosis is by clinical and radiological findings. Case Presentation: A 10-year-old female child presented in OPD with fever, wheezing and productive cough. The patient has been taking treatment for past 1 year for asthma. Chest x-ray showed reduced left lung volume, with multiple infiltrates in the left mid and lower zones. Patient was referred for a CT scan which demonstrated segmental and subsegmental bronchiectasis in the bilateral lungs, asymmetrically. Expiratory / Inspiratory CT demonstrated a significant diminution in size of the involved bronchi. William Campbell Syndrome was proposed as the underlying etiology, after exclusion of common causes of bronchiectasis. Conclusion: Bronchiectasis in children is often underdiagnosed and undertreated. When young patients present with recurrent infections, the underlying cause is important to elucidate for the management of the patient and for improving life quality and expectancy. William Campbell syndrome is a rare cause of congenital bronchiectasis due to deficient bronchial cartilage. Diagnosis in our patient was made by history, imaging and exclusion of other causes of bronchiectasis. Keywords: Bronchiectasis, High Resolution Computed Tomography Chest (HRCT Chest), William Campbell Syndrome (WCS),