Although pulmonary agenesis is an uncommon condition, it warrants consideration in all patients whose clinical course is not readily explainable on some other more satisfactory basis. This is particularly true in younger subjects. The frequency with which one encounters other malformations in patients with pulmonary agenesis has led Ferguson and Neuhauser to explain the genesis of this condition on the basis of a defect in the germ plasm. Wexels observed that nearly 50 per cent of the patients with pulmonary agenesis have other defects. He lists these anomalies as—patent ductus arteriosus, patent foramen ovale, atresia ani, atresia of the esophagus, short bowel, atresia urogenitalis, exencephaly, horseshoe kidney, ventricular defect, hypoplasia of the face, hare lip and cleft palate, absence of the left diaphragm, absent left hand, absence of right radius, bifid uvula, spina bifida, wedge-shaped vertebrae, hemivertebra, deformed ribs, accessory thumb, closed foramen epiploica, rudimentary atlas, deformed external ear, congenital ptosis of both eyelids, small left scapula, and pulmonary veins entering the azygos vein.
Pulmonary agenesis is not incompatible with life. Twenty-three patients (27 per cent) of the present series were known to have lived to be between 15 and 72 years. In reviewing the literature regarding age and agenesis, we were impressed with the fact that those who were known to have pulmonary agenesis in early life survived it only because the associated anomalies and complications were not incapacitating. Agenesis in the patient over 15 years of age is, in all likelihood, unassociated with other malformations which are of clinical significance. On the other hand, pulmonary agenesis in early life, which is clinically manifest, is often associated with other malformations which make the condition incompatible with life.
All patients who are suspected of having pulmonary agenesis should be investigated thoroughly before being subjected to any thoracic surgical procedure. A relatively minor procedure in such a patient may terminate fatally because of the lack of appreciation of the significance of the other anomalies. [see figures 3 and 4 in source pdf]