Unilateral Absence of Pulmonary Perfusion in Swyer-James Syndrome

摘要

Swyer-James syndrome is a rare disorder, usually affecting children after viral bronchitis. It is generally discovered on a chest radiograph as increased translucency involving one hemithorax because of diminished vascular markings. Bronchoscopy does not reveal obstruction, but extensive bronchitis or bronchiolitis may be observed on biopsy. The airways are normal in number, but mainly the pulmonary artery on the affected side is hypoplastic. Patency of the pulmonary artery observed on angiography differentiates this disorder from other types of pulmonary artery stenosis or atresia. The authors present a case of Swyer-James syndrome for which lung scintigraphy revealed reduced ventilation and perfusion on the affected side.