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Virtual Pediatric Hospital: Paediapaedia: Swyer James Syndrome (Unilateral Hyperlucent Lung Syndrome) Paediapaedia: Chest and Airway Diseases

Swyer James Syndrome (Unilateral Hyperlucent Lung Syndrome)

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed


Clinical Presentation:
Many are asymptomatic. Most have chronic and repeated respiratory infections, decreased exercise tolerance, arterial blood desaturation, and hemoptysis.

Etiology/Pathophysiology:
Felt to be due to adenovirus infections that are severe.

Pathology:
Necrotizing bronchiolitis with obliteration of terminal bronchioles and maintenance of peripheral alveoli.

Imaging Findings:
The initial diagnosis is made radiographically. A small or normal sized hyperlucent lung that changes little in volume during inspiration and expiration is seen. Bronchography shows bronchiectasis with failure to fill terminal airways and air sacs. Lung perfusion scintigraphy shows decreased perfusion to the involved lung. Lung inhalation scintigraphy shows delayed wash-in of radiotracer and prolonged air trapping during wash-out.

DDX:

References:
See References Chapter.

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