Scimitar syndrome

Case contributed by Dr Henry Knipe

Presentation

Shortness of breath.

Patient Data

Age: 2 days
Gender: Male
Modality: X-ray

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

Modality: CT

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 characterised 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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Case Information

rID: 41340
Case created: 27th Nov 2015
Last edited: 2nd Apr 2017
Systems: Chest, Paediatrics
Inclusion in quiz mode: Included

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