Clinical Features of Swyer-James Syndrome

Choi, Sun Hee; Kim, Do Kyun; Lee, Seon Hee; Koh, Young Yull

Abstract

PURPOSE: Swyer-James syndrome is an uncommon abnormality characterized radiographically by a hyperluncent lobe or lung and functionally by normal or reduced volume during inspiration and air trapping during expiration. The condition typically follows certain respiratory infections in infancy or early childhood. Thus, it is a postinfectious form of bronchiolitis obliterans. Most patients have chronic cough, sputum, and abnormal breathing sounds, and present with repeated pulmonary infections. The syndrome affects pulmonary functions and may cause anatomical changes such as bronchiectasis and emphysema. We reviewed cases of Swyer-James syndrome, in order to better understand the clinical manifestations and outcomes of the disease.
METHODS
The diagnostic criteria were small or normal-sized unilateral hyperlucent lung with air-trapping during expiration based on chest X-ray or CT and history of severe lung infection. A total of 12 patients were found. Medical records, radiological studies, and other clinical test results were reviewed.
RESULTS
Most of the patients had chronic respiratory symptoms and signs. All patients had pneumonia or bronchiolitis as initial insult. Microbial agents were determined in six patients, of whom three were adenovirus and the others, Mycoplasma pneumoniae. They had restrictive and obstructive patterns of pulmonary function with bronchial hyperresponsiveness for methacholine. Two patients underwent lobectomy for emphysema and bullous emphysema, respectively.
CONCLUSION
In this study, Swyer-James syndrome is a severe chronic pulmonary disease, presenting with respiratory symptoms, and functional and anatomical changes including bronchiectasis and emphysema. Further rejearch is needed to investigate initial etiologic agents and pathogenesis; further research is also needed for the care of chronic respiratory problems.