Scintigraphic images of Swyer-James syndrome

The Swyer-James Syndrome, first described in 1953, is a rare entity which consists of severe oligemia of one or more pulmonary lobes accompanied with bronchial deformation. Recurrent bronchial infections in childhood are believed to be the cause, leading to obliteration of small airways, alveolar overdistension and, ultimately, the destructive changes characteristic of emphysema. Secondary vascular alterations occur resulting in oligemia of the afflicted lobe. The clinical presentation is variable ranging from the totally asymptomatic patient to dyspnea on exertion or recurrent lower respiratory tract infections. The physical examination may reveal hyperresonance over the affected area, restricted chest expansion on one side, or occasionally rales on auscultation. Radiological findings are characteristic and include a pronounced one sided hyperlucency of the lung (due to oligemia), a mediastinal shift toward the affected side on inspiration, a small ipsilateral hilus, and bronchial deformities as disclosed by bronchography. Another radiologic feature, described as a "sine quanon" condition for diagnosis by Fraser and Paré is the development of air trapping during expiration. Various diagnostic modalities including roentgenography, bronchoscopy, bronchography, angiography and radionuclide imaging are necessary to obtain the diagnosis.