Congenital Lobar Emphysema with Dilated Septal Lymphatics

This communication reports an unusual type of congenital lobar emphysema in which the lobe was traversed by dilated lymphatics, producing a roentgen picture quite different from those seen previously. The entity of congenital lobar emphysema has received considerable attention in recent literature, particularly as a cause of respiratory distress in the newborn. Dyspnea may occur early and be rapidly progressive. More often the onset is less sudden with slowly progressive respiratory embarrassment. Jewett and Adler reviewed 40 cases in which the average age at onset was twenty-five days (6). Radiologically, the infant presents with hyperexpansion and hyperlucency of the right upper, right middle, or left upper lobes, with compression atelectasis of the remaining lobes in the hemithorax, mediastinal shift, and herniation of the involved lobe across the mediastinum (2,9). In a recent article describing the involvement of two lobes in the emphysematous process (10), emphasis is made that small lung markings will be seen crossing the hyperexpanded lung. These help to differentiate congenital emphysema from congenital cysts and pneumothorax. Pathologically, the specimen lobe is hyperexpanded and uniformly distended. On cut section the lung tissue presents a spongy appearance with multiple distended alveoli, typical of hypertrophic pulmonary emphysema (1). While in some cases lack of bronchial cartilage or a flap of mucosa is a possible etiological factor for the emphysema, in many others no such cause is evident. It has been suggested that in some patients a bronchial defect might have been present but not included in the portion removed at surgery (10). The following case was clinically quite usual, but both roentgen and pathological findings differed from any observed at our hospital or reported in the literature. Case Report A ten-hour-old white male born Nov. 27, 1964, was admitted to Denver Children's Hospital in respiratory distress which began shortly after birth and progressively increased in severity. Birth weight was 7lb. 12 oz. On physical examination the infant appeared dusky to cyanotic. The chest showed an increased anteroposterior diameter, left greater than right, with expiratory wheezes bilaterally. A chest film on admission revealed a grossly hyperexpanded left upper lobe with shift of the mediastinum to the right and almost total atelectasis of the left lower lobe. Instead of being hyperlucent, however, the hyperexpanded left upper lobe was abnormally dense, mostly because of a network of linear shadows which traversed the entire lobe seemingly without pattern. Minimal patchy density was also present (Fig. 1). Radiographic diagnosis was congenital lobar emphysema. The linear densities could not be explained. A left upper lobectomy was performed, and the patient recovered uneventfully. Pathology The specimen was the left upper lung lobe with a lingula.