Scimitar syndrome

Case contributed by Henry Knipe


Shortness of breath.

Patient Data

Age: 2 days
Gender: Male

Mediastinal shift to the right with near white-out of the right hemithorax. 


Hypoplastic right lung with mediastinal shift to the right and basal opacity. There is an anomalous pulmonary vein draining to the inferior vena cava. Systemic arterial supply to the right lower lobe is also demonstrated.

ETT tip is well above the carina. Mild cardiomegaly. No pleural or pericardial effusion. Hypoplastic right pulmonary artery.

Case Discussion

Scimitar syndrome (a.k.a. pulmonary venolobar syndrome or hypogenetic lung syndrome) is characterized by pulmonary hypoplasia that is drained by an anomalous vein into the systemic venous system. There is an uncommon association with lung sequestration (~10%). 

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