Swyer-James syndrome

Case contributed by Yune Kwong
Diagnosis almost certain

Presentation

Symptoms of asthma.

Patient Data

Age: 25 years old
Gender: Female
x-ray

CXR shows a paucity of vascular markings in the left mid and lower zones.

ct

On inspiratory views, bronchiectasis is seen in the left lower lobe which is associated with volume loss (compare the position of the oblique fissures at the bases). There is also subtle hyperlucency of the left lower lobe, with associated small caliber vessels. This is suggestive of air trapping, which is confirmed on expiratory HRCT. Note also that there are small lobular areas of air trapping in the right lung.

Case Discussion

Swyer-James (also known as MacLeod) syndrome is a form of bronchiolitis obliterans, which occurs secondary to injury to the immature lung. The lung served by the damaged airways is aerated by collateral air drift through the pores of Kohn. It typically presents as an incidental finding on CXR performed for unrelated reasons.

On CXR, the affected lung is of small or normal volume. Unilateral transradiancy is detected in severe cases. The affected side shows a reduction in size and number of pulmonary vessels (with reduction in size of the hilum), whilst the contralateral side looks plethoric.

HRCT additionally demonstrates that the findings of air trapping are often bilateral (CXR is not sensitive enough to demonstrate bilateral changes). Bronchiectasis is a common additional finding.

The most important differential to exclude is a central obstructing lesion causing air trapping.

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