AGENESIS OF THE LUNG

Publisher Website
Google Scholar
Abstract
One hundred sixty-four cases of agenesis of the lung are reviewed, including 36 not previously reviewed and 8 not previously reported. Agenesis, affecting both hemithoraces and both sexes almost equally, should be separated for physiologic reasons from hypoplasia by the total absence of lung parenchyma, bronchial tree, as well as supporting vasculature. Genetic, teratogenic, and mechanical factors may have a bearing on etiology. Chest roentgenograms suggest the diagnosis initially, but careful bronchographic and angiocardiographic studies are necessary for confirmation. The electrocardiogram is helpful in the differential diagnosis of the patients with dextrocardia. Severe respiratory infections are common in infancy and may lead to pneumonia and death. The condition, however, is not uniformly fatal; 24 of 36 patients reported since 1954 were alive at the time of reporting. There is no evidence at present that resting pulmonary artery hypertension or emphysema develops in patients with agenesis of the lung and an intact cardiovascular system. Diagnostic studies reveal pulmonary artery hypertension in a number of patients with a combination of systemicpulmonary communication and agenesis of the lung. Coexisting abnormalities may involve the skeletal, cardiovascular, gastrointestinal, and genitourinary systems.

This publication has 0 references indexed in Scilit: