Respiratory problems of early life now allowing survival into adulthood: Concepts for radiologists

Abstract

Many patients with illnesses that once were fatal at birth or during childhood now survive into adult life. This article considers four respiratory illnesses of early life in which long-term survival now occurs frequently: cystic fibrosis, diaphragmatic hernia, esophageal atresia-tracheoesophageal fistula, and bronchopulmonary dysplasia. In cystic fibrosis, although the median age at death is now 25 years, chronic pulmonary infection due ultimately to the abnormal composition and clearance of airway mucus is still the usual cause of death. Earlier survivors of congenital diaphragmatic hernia had only minor diminution of perfusion and ventilation of the lung on the side of the hernia as adolescents or young adults; however, as infants with greater degrees of pulmonary hypoplasia have successful repair of their hernias, more long-term respiratory impairment will probably be found. The esophageal atresia-tracheoesophageal fistula complex leaves all esophagi and many tracheas permanently abnormal; recurrent aspiration, repeated pneumonia, and an unduly collapsible trachea are the result, although symptoms may be few. Survivors of bronchopulmonary dysplasia have decreased exercise capacity, wheezing, and recurrent pneumonia, although their chest radiographs may become normal or almost normal.