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Scimitar Syndrome with Dilated Azygos Vein and Interrupted Inferior Vena Cava

Theresa NH Leung and Na Xie

A 3 year-old girl with recurrent chest infections had chest radiograph showing the typical “Turkish sword” sign which could be found in 70% in the childhood-adult group of Scimitar syndrome, a rare form of partial anormalous pulmonary venous drainage (PAPVD) associated with hypoplastic lung. The anomalous pulmonary veins drain into the systemic venous system such as inferior vena cava (IVC), right atrium, hepatic or portal vein.