Scimitar syndrome

Case contributed by Melbourne Uni Radiology Masters


Pre operative CXR.

Patient Data

Age: 40 years
Gender: Male

Chest radiographs

Heart size is within normal limits. Hypoplastic right lung with linear opacity consistent with venous drainage at the right lung base in keeping with known scimitar syndrome. The left lung is clear. No acute osseous abnormality.

Case Discussion

Scimitar syndrome, also known as pulmonary venolobar syndrome or hypogenetic lung syndrome, is characterized by a hypoplastic lung that is drained by an anomalous vein into the systemic venous system. It is a type of partial anomalous pulmonary venous return.

It is essentially a combination of pulmonary hypoplasia and partial anomalous pulmonary venous return (PAPVR). It almost exclusively occurs on the right side.

Haemodynamically, there is an acyanotic left to right shunt. The anomalous vein usually drains into: 

  • inferior vena cava: most common 
  • right atrium 
  • portal vein


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Case information

rID: 42677
Published: 9th Feb 2016
Last edited: 14th Aug 2019
System: Chest
Tag: rmh
Inclusion in quiz mode: Included

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