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Congenital hypogenetic lung syndrome

Case contributed by Hani Makky Al Salam
Diagnosis certain

Presentation

Asymptomatic

Patient Data

Age: 30 years
Gender: Female

The plain chest radiograph shows a prominent anomalous pulmonary vein traveling parallel to the right wall of the heart and disappearing in the right cardiophrenic angle. There is small right lung and mediastinum shift to the right side with cardiac dextroposition. There is reduced right lung vascularity.

The CT confirm the presence of the anomalous vein draining into the supradiaphragmatic inferior vena cava.

Case Discussion

Hypogenetic lung syndrome, also known as scimitar syndrome, is a group of disorders of lung and vascular development that occur together. The most consistent features of hypogenetic lung syndrome are anomalous pulmonary venous return of the right lung, which may be partial or complete and pulmonary hypoplasia of the right lung ranging from mild to severe. 

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