CTICasesSwyer-James Syndrome
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Imaging findings
A chest radiograph revealed scoliosis and lucency of the left lung base, initially suggesting emphysema. Centrally, a 'cluster of grapes' pattern of dilated bronchi was evident, also present on the right, consistent with widespread bronchiectasis. Lunules of pus or sputum were seen within the bronchiectatic airways. The main pulmonary artery was dilated, indicating pulmonary hypertension. An outside CT scan confirmed widespread bronchiectasis and extensive mosaic attenuation, suggestive of air trapping (though an expiratory sequence was not performed).
Key takeaways
This 40-year-old Romanian man presented with 15 years of dyspnea. Initial clinical evaluation for cystic fibrosis, ciliary dysmotility, and immune deficiency was negative, despite a childhood history of severe respiratory infection. The pulmonologist initially dismissed Swyer-James syndrome because it was considered a unilateral disease, whereas the patient had bilateral findings. However, radiologists noted that Swyer-James syndrome is typically patchy and involves both lungs on CT. The diagnosis was converted to Swyer-James syndrome, with the childhood infection (likely adenovirus) as the precipitating event, leading to obliterative bronchiolitis and associated bronchiectasis.
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See all cases from April 11, 2014 →