A Case of Swyer-James Syndrome, with Reference to Pathophysiologic and Etiologic Aspect

A 21-year-old male of Swyer-James syndrome is presented with clinical, angiographic, bronchographic and scintigraphic studies. He had a past history of recurrent bronchial asthma. A chest roentgenogram revealed unilateral hyperlucency of the left lung without any changes in volume. The small size of the pulmonary artery and its branches of the left lower lobe was visible by pulmonary angiography. Bronchography showed the bronchiectatic changes which consisted of moderate bronchial dilatation in an irregular beaded pattern with clubbed or tapered ends accompanied by absence of alveolar filling. A perfusion lung scan using 99mTc-macroaggregated albumin (99mTc-MAA) illustrated a perfusion defect in the left lower lobe. It was thought that recurrent pulmonary infections and asthmatic attacks were responsible for a pathological condition of this disease.