Scimitar syndrome

Case contributed by Mohammad Farghali Ali Tosson


Right sided chest pain. Incidental finding.

Patient Data

Age: 30 years
Gender: Male

Right well defined supradiaphragmatic hypoattenuated non-enhancing 'pseudomass' measuring about 6 x 4 x 4 cm with abnormal aberrant small arterial supply from the abdominal aorta.

The right lung volume is reduced (hypoplastic).

The venous drainage is via the dialted single right pulmonary vein into the left atrium.  The right sided anomalous pulmonary vein drains into the IVC as part of the PAPVR,

Bilateral left sided branching of the bronchial tree.

Case Discussion

This is a classic case of scimitar syndrome (hypogenetic lung syndrome), which is a combination of:

  • partial anomalous pulmonary venous return (PAPVR) in the right lower lobe with a dilated tortuous vein draining into the left atrium
  • hypoplastic right lung with a small feeding artery from the abdominal aorta

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