IS Case 146: Scimitar syndrome (Congenital Venolobar or Congenital Hypogenetic Lung syndrome)

Daniel Ginat, MD, MS

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Imaging Sciences URMC 2008
Publication Date: 2009-05-21

History

Patient is a 47-year-old-female status post-patent foramen ovale (PFO) closure.

Findings

Chest X-ray: There is a right lower lung zone vertical density that parallels the right heart border. There is also a hypoplastic right lung with mild rightward mediastinal shift.

CT: There is anomalous pulmonary venous drainage into the inferior vena cava.

Diagnosis

Scimitar syndrome (Congenital Venolobar or Congenital Hypogenetic Lung syndrome)

Discussion

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.

References

1. Berrocal T, Madrid C, Novo S, et al. Congenital anomalies of the tracheobronchial tree, lung, and mediastinum: embryology, radiology, and pathology. Radiographics. 2004 Jan-Feb;24(1):e17. [PMID: 14610245]
2. Ferguson EC, Krishnamurthy R, Oldham SA. Classic imaging signs of congenital cardiovascular abnormalities. Radiographics. 2007 Sep-Oct;27(5):1323-34. [PMID: 17848694]