Progressive stridor: could it be a congenital cystic lung disease?


M Zedan, M.D., Allergy, Respiratory and Clinical Immunology Unit, Mansoura Faculty of Medicine, Mansoura University, Mansoura, Egypt. Tel: +20502242526 | Fax: +20502234092 |


Bronchogenic cyst of the mediastinum, a cause of stridor early in life, is the result of abnormal budding of the ventral segment of the primitive foregut. Bronchogenic cysts are often asymptomatic in older children and adults. However, symptomatic cases usually manifest early in life with cough, stridor or wheezing due to airway compression. We report a female infant aged 4.5 months with a normal full-term pregnancy, who developed respiratory distress with stridor. This stridor was preceded by a history of slowly progressive noisy breathing. Physical examination revealed evidence of bilateral obstructive emphysema. Chest radiograph revealed bilateral overinflation. Fibro-optic bronchoscopy revealed posterior mediastinal compression. Possibility of congenital cystic lung disease (CCLD) was considered, emphasizing the value of computed tomography (CT) chest, which revealed a cyst probably bronchogenic. Surgical excision was performed with evident histological confirmation of bronchogenic cyst.

Conclusion:  we highlight that in any infant, presented with slowly progressive noisy breathing in the first year of life, CCLD should be considered in the differential diagnosis even with normal X-ray chest. CT chest should be performed for exclusion or diagnosis of the case.