A case of Swyer-James syndrome diagnosed at age 70

A male case of Swyer-James syndrome, who had been followed for bronchiectasis for a long time, detected at age 70 and diagnosed by radiological studies was reported. The movement of his left diaphragm was impaired. Arterial oxygen tension was 68 torr while breathing room air. Pulmonary function tests showed mixed ventilatory impairment, increase of residual volume, and uneven gas distribution, but diffusing capacity was normal with the single breath method. Chest radiograph revealed hyperlucency of the left lung and the right middle lung field. In these areas, pulmonary angiography showed marked hypovascularity and diffuse vascular atrophy, and perfusion and inhalation scans also showed marked decreased perfusion and ventilation. Pulmonary hypertension was not detected by right heart catheterization. Fiberoptic bronchoscopy was performed and showed no abnormality of the left bronchial tree, and bronchography demonstrated diffuse cylindrical bronchiectasis of the left lung with no alveolar filling. He had suffered from chronic sinusitis for a long time, which might suggest that he had sinobronchitis due to congenital abnormality of the airway and this caused continuous respiratory infection, which could cause this syndrome. Including our own case, about 80 cases of Swyer-James' syndrome have been reported in Japan. But only 4 cases were above 70 years of age and all of them were male, and there has been no female cases above age 50. Males predominate over females, and in both sexes the left lung was more commonly affected than the right.