hypoplasia / aplasia of one or more lobes of lung
always on RIGHT
+/- partial APVR (to IVC, portal vein, hepatic vein.)
scimitar-shaped density is draining vein
- one manifestation of a more generalized disorder called congenital pulmonary venolobar syndrome. This syndrome encompasses a number of congenital abnormalities including: hypogenetic lung, partial anomalous pulmonary venous return, absence of pulmonary arteries, pulmonary sequestration, absence of IVC, and accessory diaphragm. When hypogenetic lung and partial anomalous pulmonary venous return coexist, it is termed scimitar syndrome. The anomalous draining pulmonary vein most commonly drains into the IVC, but may on occasion drain into the hepatic veins, portal veins, azygous vein, coronary sinus, or right atrium. The abnormality is almost always right sided. Associated anomalies include branchial isomerism and anomalous arterial supply to the hypogenetic lung (usually systemic arteries such as bronchial).