Congenital venolobar syndrome

  • Scimitar syndrome
  • Lung hypoplasia and ipsilateral anomalous systemic venous drainage
  • Anomalous right pulmonary vein drains into IVC, hepatic or portal vein, coronary sinus or right atrium
  • Most asymptomatic
  • Left-to-right shunt  can lead to pulmonary hypertension
  • Similar to isolated lung hypoplasia – increased opacity of one hemithorax, mediastinal shift, contralateral lung hyperinflation
  • Anomalous vein – tubular shadow, runs to base of lung, resembles a curved sword (scimitar)
  • CECT: best technique
Read more

Neal, Kevin A., and Marilyn J. Siegel. “The Hypogenetic Lung (Scimitar) Syndrome.” Radiology 293.3 (2019): 522-522.

Cirillo Jr, Robert L. “The scimitar sign.” Radiology 206.3 (1998): 623-624.