What conditions could explain the appearance of this chest X-ray?
- Left pneumothorax
- Hiatus hernia
- Right hypoplastic lung
- Right pulmonary hypoplasia
- Scimitar syndrome
What are the most appropriate investigations?
- Ventilation scan
- Bronchoscopy
- Echocardiogram
- Barium swallow and meal
- CT lung scan
1 Comments
Answers: (a) 3, 4, 5
(b) 3, 5
Scimitar syndrome
A diagnosis of scimitar syndrome was eventually made on this girl after referral to a pediatric cardiologist. The ‘scimitar’-shaped shadow seen on the chest X-ray is an anomalous right pulmonary vein draining into the inferior vena cava. Following further investigations, which included a chest CT scan (with contrast), an echocardiogram, and a cardiac catheter study that included pulmonary and aortic angiograms, she was found to have a hypoplastic right lung contributing only 30% of the total ventilation and 1% of the perfusion. No anomalous vessels
were found arising from the aorta. Her condition is not thought to be operable. As with other individuals, she will be prone to lifelong recurrent chest infections and may develop bronchiectasis when older. Management is supportive, with chest physiotherapy and prophylactic antibiotics.