Generalized Pulmonary Emphysema as an Isolated Manifestation of Early Cystic Fibrosis of the Pancreas

Abstract

The pulmonary manifestations of advanced cystic fibrosis of the pancreas are now generally well recognized. This is not true, however, of the earliest roentgenologic signs, though it is of great importance that these be recognized, so that treatment may be instituted to postpone the later irreversible changes. Generalized pulmonary emphysema as an isolated manifestation of the earliest phase of pulmonary involvement is frequently seen and, unless one is aware of the existence of this entity, delay in correct diagnosis may result. It is the purpose of the present report to describe this manifestation and to emphasize its importance. The pathologic changes in the lungs of patients with pancreatic cystic fibrosis are considered to be the result of bronchial obstruction produced by the tenacious secretion which characterizes this disease. Neu-hauser (1) has pointed out that the pulmonary changes occur in two stages. First, there are various degrees of bronchial obstruction without infection, which result in obstructive emphysema with poor gaseous exchange. The emphysema is quite irregular and one or more areas of plate-like atelectasis or lobar collapse may be seen. In the second stage, infection is superimposed, and there are multiple areas of peribronchial pneumonia. Later, bronchiectasis, atelectasis, and bronchiectatic abscesses supervene. While it is generally agreed that emphysema and atelectasis are cardinal manifestations of early cystic fibrosis of the pancreas, most reports have been concerned with the irregularity of the emphysema and its association with atelectasis (2–6). However, generalized emphysema alone is not uncommon, and to the uninitiated may present a diagnostic problem if its significance is not understood. This is particularly true since the roentgen picture early in the disease is normal and the emphysema may be overlooked or disregarded if it is not of a marked degree. The later states of atelectasis and infection follow the emphysema in varying periods of time, usually short, but occasionally prolonged. Roentgenographically, generalized pulmonary emphysema is not always easily recognized in children. This is due, for the most part, to difficulty in obtaining films in the proper phase of respiration. In order to gain a correct impression of the state of aeration of the lungs, it is important to make several exposures in the anteroposterior plane, preferably in inspiration and expiration, and to obtain at least a single lateral projection as well. In children generalized emphysema may manifest itself by increase in the anteroposterior diameter of the chest, particularly increased width of the retrosternal and retrocardiac spaces, by elevation of the anterior ends of the ribs, flattening of the domes of the diaphragm, increased radi-ability of the lungs with paucity of parenchymal markings, and diminished size of the cardiac silhouette.