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

• Published in: Acta Paediatrica Vol. 98; no. 9; pp. 1533 - 1536
• Main Authors: Zedan, M, Elgamal, MA, Zalata, K, Nasef, N, Fouda, A
• Format: Journal Article
• Language: English
• Published: Oxford, UK Blackwell Publishing Ltd 01.09.2009; Blackwell
• Subjects: Airway Obstruction - etiology; Biological and medical sciences; Bronchogenic Cyst - complications; Bronchogenic Cyst - diagnosis; Bronchogenic cysts; Bronchoscopy; Diagnosis, Differential; Female; General aspects; Humans; Infant; Medical sciences; Progressive stridor; Pulmonary Emphysema - complications; Pulmonary Emphysema - diagnosis; Respiratory Insufficiency - etiology; Respiratory Sounds - etiology; Sensitivity and Specificity; Tomography, X-Ray Computed; Progressive; Stridor; Lung disease; Pediatrics; Skin disease; Respiratory disease; Bronchogenic cyst; Bronchogenic cysts; Progressive stridor; Congenital disease
• ISSN: 0803-5253; 1651-2227
• DOI: 10.1111/j.1651-2227.2009.01366.x

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.