Congenital lobar emphysema has been described under various designations. Although the etiology is not clear, some form of bronchial obstruction has been documented in about 50% of the patients. A rare case of congenital lobar emphysema with a membranous septum in the left main bronchus is described. Although prompt lobectomy has been the treatment of choice in most cases, this case demonstrates the importance of early bronchoscopy to exclude treatable intra luminal abnormalities.
An eight-week-old infant presented with dyspnoea two months after an uneventful normal vaginal delivery. Radiologically, a sharply outlined radiolucent area surrounded by atelectasis was seen in the upper lobe of the left lung. A left upper lobectomy was performed with the clinical impression of congenital pulmonary emphysema. The resected specimen displayed multiple cysts 2 to 6 mm in diameter. Microscopically, intracystic papillary mesenchymal ingrowths lined by respiratory epithelium were present. Based on both the gross and microscopical features, a diagnosis of Fisher's variant of type II congenital cystic adenomatoid malformation (CAM) was made. The postoperative follow-up showed excellent recovery and normal development of the child.