Scimitar Syndrome and Associated Pulmonary Sequestration

Abstract

The scimitar syndrome is characterized by anomalous venous drainage from the right lung to the vena cava inferior. The appearance of this vein on chest X-ray is suggestive of a Turkish sword and is called the “scimitar” sign by Neill et al. (1960). The condition may be associated with anomalies of the right lung (absence of lung fissures, mirror image of the left lung, absence of lobe bronchi), anomalies of the pulmonary artery, and congenital heart defects (Mathey et al. 1968; Mende et al. 1973). The symptoms are similar to those of an atrial septal defect due to a left-to-right shunt, such as repeated respiratory infections. We present an uncommon case of scimitar syndrome combined with pulmonary sequestration; few cases of this anomaly have been reported in the literature (Alivizatos et al. 1985; Ohsaki 1983).