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Scimitar Syndrome


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Scimitar Syndrome

Elliot K. Fishman, MD
Director of Diagnostic Imaging and Body CT, Professor of Radiology and Radiological Science, Johns Hopkins
Karen M. Horton, MD
Professor and Interim Chair, Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins

This is a 19 year old male presenting with cough. Frontal and lateral radiographs of the chest were obtained. Initial evaluation of the frontal radiograph demonstrates asymmetric inflation of the lungs, with the right lung appearing less expanded than the left lung. The right lung is hypoinflated. Additionally, the red arrow points to a vertically oriented tubular structure. On the lateral radiograph, this tubular structure is again identified by the red arrow. The green arrow corresponds to a hyperlucent left lower lobe secondary to compensatory hyperinflation. Findings are consistent with Scimitar syndrome. The term "Scimitar" refers to the fact that the vertically oriented tubular structure resembles the Turkish sword known as a scimitar. In this condition, a portion of the right lung is hypoplastic and is drained by an anomolous vertically oriented pulmonary vein. Most commonly, this vessel drains into the inferior vena cava instead of the left atrium.