IS Case 146: Scimitar syndrome (Congenital Venolobar or Congenital Hypogenetic Lung syndrome)
Imaging Sciences URMC 2008
Publication Date: 2009-05-21
Scimitar syndrome consists of a complex of abnormal blood supply to the right lung, pulmonary hypoplasia, abnormal bronchial anatomy, abnormal diaphragm, hemivertebrae, and anomalies of the genitourinary tract. The most invariable feature of Scimitar syndrome is anomalous venous drainage, usually by a single vein for the entire right lung. Rarely, the left lung is affected.
The classic radiographic finding is that of a right pulmonary vein descending along the right cardiac border in a configuration of a Turkish sword, known as "scimitar sign". Angiography can reveal the anomalous venous drainage and is useful for obtaining a measure of the right pulmonary artery. Contrast-enhanced MR or CT can confirm the diagnosis and demonstrate additional associated abnormalities.
About 40% of patients with scimitar syndrome are asymptomatic. The remainder may experience dyspnea, fatigue, and pulmonary infections. Prognosis depends largely on the extent of left-to-right shunting.
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