Scimitar syndrome

Case contributed by Anil Kumar Geetha Virupakshappa
Diagnosis certain

Presentation

Cough for 1 week duration

Patient Data

Age: 35 years
Gender: Female
x-ray

Chest X-ray shows volume loss or small right hemithorax with ipsilateral mediastinal shift, elevated right dome of diaphragm and obscuration of right heart border and right hemidiaphragm.

ct

There is partial anomalous pulmonary venous return to IVC, best seen on coronal images with an appearance similar to a Turkish sword or scimitar.

There is non-development of the right upper lobe and its bronchus with associated volume loss, mediastinal shift, elevated right dome of diaphragm and compensatory hyperinflation of the right middle and lower lobes.

Case Discussion

Scimitar syndrome is a type of partial anomalous pulmonary venous return from a hypoplastic lung to the systemic circulation, most commonly to the inferior vena cava, causing a left-to-right shunt.

When discovered as an incidental finding in an adult, the prognosis is typically benign. Shunts exceeding 50% can cause pulmonary hypertension or heart failure and should be surgically corrected.

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