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A 29 year old male, previously fit, presented with cough, breathlessness, and pyrexia. He had depression and reduced movement of the left side of the chest. There was dullness and absent breath sounds at the upper left chest area. The apex beat, however, was in the second left intercostal space. The posteroanterior chest film (left panel) showed that the cardiac silhouette was not in the normal position but displaced to the upper left thorax. A computed tomographic scan confirmed that the heart lay within the left upper thorax with the apical segment of the left lower lobe of the lung seen to lie posterior to the heart. At bronchography the left main bronchus divided into a normal left lower bronchial tree, but the upper left bronchus terminated abruptly indicating left upper lobar aplasia (right panel). A transthoracic echocardiogram recorded at the second last interspace showed no intracardiac abnormality. His symptoms were caused by a viral upper respiratory infection and he made a full recovery.
Agenesis of the lung may be bilateral, unilateral or lobar. There may be associated cardiac and skeletal abnormalities. Flattening of the thorax and crowding of the ribs of the affected area is common. About half of affected individuals die in the first year of life but survival to old age has been recorded.
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