Case Author(s): Gregg Schubach/F. Dehdashti , 9/8/95 . Rating: #D2, #Q4

Diagnosis: Swyer-James syndrome

Brief history:

7-year old boy with history of previous pneumonia.


one minute sequential ventilation images

View main image(vq) in a separate image viewer

View second image(vq). perfusion images

View third image(xr). PA Chest Radiograph (contrast adjusted to accentuate lung markings)

Full history/Diagnosis is available below

Diagnosis: Swyer-James syndrome

Full history:

7-year old boy with history of previous pneumonia. The patient1s recent chest radiograph demonstrates diminished vascularity in the left lower lung zone. The patient is currently asymptomatic. The clinical and radiographic findings are suggestive of Swyer-James syndrome. Ventilation-perfusion scintigraphy was obtained to assist in confirming this diagnosis.


The Xe-133 washin ventilation images demonstrate hypoventilation of the left lower lung. The washout images show Xe-133 retention in this region. This abnormality corresponds to the region of radiographic abnormality. The perfusion images show hypoperfusion in the left lower lung, corresponding to both the ventilation and radiographic abnormalities.


Swyer-James syndrome (Macleod syndrome, unilateral hyperlucent lung) is likely secondary to childhood adenoviral infection with subsequent acute obliterative bronchiolitis developing between 7-30 months post infection. At the time of diagnosis, the patients are usually asymptomatic but may experience dyspnea on exertion. There is almost invariably a history of repeated lower respiratory tract infections during childhood. Swyer-James syndrome is typically described as involving one lung; however, it may occur in various anatomical distributions, including one lobe, two lobes in one lung and may affect both lungs. The radiographic findings include increased radiolucency of the affected region and small hemithorax with either decreased or normal volume. However, the sine qua non for diagnosis is the presence of air trapping during expiration. Nuclear medicine imaging classically demonstrates decreased ventilation with delayed washout and decreased perfusion. Angiography, although rarely necessary, demonstrates diminutive hilar vessels on the affected side with narrowed, attenuated arteries coursing through the radiolucent lung (the so-called "pruned tree appearance").

References: Daniel TL, Woodring JH, Vandiviere HM, et al.: Swyer-James syndrome- unilateral hyperlucent lung syndrome. A case report in review. Clinical and Geriatrics 1984;23:393. O1Dell CW, Taylor A, et al.: Ventilation-perfusion lung images in the Swyer-James syndrome. Radiology 1976;121:432-426. Fraser et al.: Diagnosis of diseases of the chest. 3rd Ed.


The clinical, radiographic, and scintigraphic findings are considered pathognomonic. No further follow-up was deemed necessary.

Major teaching point(s):

Although the clinical and radiographic findings are often diagnostic, radionuclide ventilation-perfusion scintigraphy may reveal typical findings of Swyer-James syndrome in the presence of subtle radiological changes or additional areas of involvement in regions which are radiographically normal.

ACR Codes and Keywords:

References and General Discussion of Ventilation Perfusion Scintigraphy (Anatomic field:Lung, Mediastinum, and Pleura, Category:Organ specific)

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Case number: vq011

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