CTICasesScimitar Syndrome (Hypogenetic Lung Syndrome)
Imaging findings
High-resolution CT shows anomalous right pulmonary venous drainage, where a large, anomalous "scimitar" vein courses inferiorly to drain into the inferior vena cava (IVC), while other veins drain normally into the left atrium. There are associated airway anomalies, including hypoplastic and abnormal bronchial branching patterns in the right lung.
Key takeaways
Scimitar syndrome, or hypogenetic lung syndrome, is a form of partial anomalous pulmonary venous return (PAPVR) characterized by an anomalous right pulmonary vein draining into the IVC, right lung hypoplasia, and bronchial anomalies. While frequently associated with systemic arterial supply from the aorta, the syndrome can present as a spectrum of vascular and bronchial anomalies without systemic arterialization.
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