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Joseph Junewick, MD FACR
over 8 years ago
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Scimitar Syndrome

Case Detail

Anatomy: Chest
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Vascular
Created: over 5 years ago
Updated: over 5 years ago
Tags: PEDS
Modality/Study Types: CR CT
Activities:
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History

4 year old male with dyspnea and asthma-like symptoms.


Case Images


Diagnosis

Scimitar Syndrome

Findings

CR – Chest radiograph shows decreased aeration of the right hemithorax and displacement of the heart and mediastinum to the right. There is loss of the right heart shadow due to the right pulmonary hypoplasia. The anomalous right sided pulmonary vein extending inferomedially forms the “scimitar”.

CT – Axial image with lung windows confirms right pulmonary hypoplasia. Oblique coronal reformatted image shows the scimitar vein emptying into the supradiaphragmatic inferior vena cava.

Discussion

The lungs initially derive systemic arterial and venous supply from the cardinal system. Later the common pulmonary vein develops from the primitive left atrium and grows toward the developing lung to re-establish venous connections. When this process fails, anomalous venous drainage occurs.

Anomalous pulmonary venous return results from the persistence of the pulmonary vein connections to the cardinal systemic veins – this may be total or partial. One form of partial anomalous pulmonary venous return is the scimitar syndrome where the right pulmonary veins drain to the inferior vena cava.

Scimitar syndrome is associated with right lung hypoplasia and subsequently the heart is often dextropositioned. The right lower lobe may have a pseudosequestration appearance. The right pulmonary artery is small and there are often multiple aorto-pulmonary collaterals.

Reference

Vyas HV, Greenberg SB, Krishnamurthy R. MR imaging and CT Evaluation of congenital pulmonary vein abnormalities in neonates and infants. Radiographics (2012); 32:87-98.



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