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A 7 year old boy was admitted with severe respiratory distress. A chestx ray film showed diffuse opacity of the right hemithorax and a slightly overdistended left lung with patchy infiltrates; the cardiac silhouette was shifted to the right (fig 1). Despite treatment with intravenous antibiotics, inhaler steroids, and bronchodilators, the patient's radiograph did not clear. A flexible bronchoscopy was carried out to determine whether a foreign body had been aspirated. The right bronchus was narrow and the catheter could not be passed into it (fig 2). Right pulmonary agenesis was therefore considered. No right pulmonary artery and vein or any lung tissue on the right was visualised at pulmonary angiography (fig3).
In the presence of an opaque hemithorax with ipsilateral cardiomediastinal shift and an intact diaphragm on the radiograph, pulmonary agenesis should always be considered in the differential diagnosis, which includes diaphragmatic hernia, adenomatoid cystic malformation, and sequestrations. Bronchoscopy was used here to show narrowing of the main bronchus, but the best method for precise diagnosis of pulmonary agenesis is pulmonary angiography, which confirmed the absence of the ipsilateral pulmonary vessel.