eMedicine Specialties > Radiology > Chest

Swyer-James Syndrome: Imaging

Author: Beverly P Wood, MD, MS, PhD, Professor, Departments of Radiology and Pediatrics, Division of Medical Education, Keck School of Medicine, University of Southern California
Contributor Information and Disclosures

Updated: Sep 25, 2008

Radiography

Findings

  • The typical appearance of Swyer-James syndrome is that of a hyperlucent but small lung, with overexpansion of the contralateral lung.
  • A comparison of progressive radiographs shows failure of growth in the involved lung.
  • A diffuse pattern of scarring or irregular vessels may be present.
  • Fluoroscopy shows little change in volume in the involved lung with respiration (see Images 1-6).

Degree of Confidence

The disparity in size between the 2 lungs may represent a hypoplastic pulmonary artery or congenital hypoplasia of the lung. A comparison with a previous set of radiographs helps with the differential diagnosis. A history of severe lung infection also helps in making the diagnosis.

Computed Tomography

Findings

In Swyer-James syndrome, the bronchi have a pruned appearance. A mosaic pattern of air trapping in acini is seen, along with air trapping during expiration. The appearance is similar to that of hypoplastic lung syndrome.11,12

False Positives/Negatives

The appearance of Swyer-James syndrome is the same as that of bronchiolitis obliterans, but bronchiolitis obliterans is more frequently a diffuse process.

Magnetic Resonance Imaging

Findings

In Swyer-James syndrome, the pulmonary vessels of the affected lung appear smaller than normal on MRI. Peripheral branches of the pulmonary vessels do not develop, and vasculature is arrested at the stage at which the infection occurred.

Ultrasonography

Findings

Ultrasound is usually not useful in the assessment of the patient with Swyer-James syndrome.

Nuclear Imaging

Findings

On ventilation-perfusion lung scanning, diminished activity of the affected lung is seen with perfusion scanning, and decreased gas exchange is seen during the ventilatory phase. The lung perfusion deficit seen in patients with Swyer-James syndrome occurs because the peripheral branches of the pulmonary vessels have not developed normally, and vasculature is arrested at the stage at which the causative infection occurred.

False Positives/Negatives

Any disorder involving distal airway obstruction (ie, bronchiolitis obliterans, asthma, congenital lobar emphysema) may present in the same manner as Swyer-James syndrome.

Angiography

Findings

In patients with Swyer-James syndrome, the pulmonary artery and its branches are small and hypoplastic on the involved side. Collateral vessels may be present, but they are unusual.

Degree of Confidence

With angiography, acquired hypoplastic lung cannot be differentiated from congenital hypoplasia of the lung.

False Positives/Negatives

Swyer-James syndrome is similar in appearance to lobar emphysema, congenital hypoplasia of the lung, and hypoplastic pulmonary artery.

More on Swyer-James Syndrome

Overview: Swyer-James Syndrome
Imaging: Swyer-James Syndrome
Follow-up: Swyer-James Syndrome
Multimedia: Swyer-James Syndrome
References
Further Reading
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References

  1. Braunschweig M, Gal I. Swyer-James syndrome. JBR-BTR. Apr 2001;84(2):57. [Medline].

  2. Chalmers JH Jr. Swyer-James syndrome. Semin Respir Infect. Sep 1999;14(3):295-7. [Medline].

  3. Cumming GR, Macpherson RI, Chernick V. Unilateral hyperlucent lung syndrome in children. J Pediatr. Feb 1971;78(2):250-60. [Medline].

  4. Khalil KF, Saeed W. Swyer-James-MacLeod Syndrome. J Coll Physicians Surg Pak. Mar 2008;18(3):190-2. [Medline].

  5. Gopinath A, Strigun D, Banyopadhyay T. Swyer-James syndrome. Conn Med. Jun-Jul 2005;69(6):325-7. [Medline].

  6. Górska L, Kuziemski K, Wajda B, Damps-Konstanska I, Tokarska B, Jassem E. [Hyperlucent lung syndrom caused by pulmonary artery hypoplasia in patient with diagnosed asthma--case report]. Pol Merkur Lekarski. May 2008;24(143):436-8. [Medline].

  7. Kiratli PO, Caglar M, Bozkurt MF. Unilateral absence of pulmonary perfusion in Swyer-James syndrome. Clin Nucl Med. Sep 1999;24(9):706-7. [Medline].

  8. Lucaya J, Gartner S, Garcia-Pena P. Spectrum of manifestations of Swyer-James-MacLeod syndrome. J Comput Assist Tomogr. Jul-Aug 1998;22(4):592-7. [Medline].

  9. Kim CK, Koh JY, Han YS, Kang H, Kim JS, Koh YY. Swyer-James Syndrome with finger clubbing after severe measles infection. Pediatr Int. Jun 2008;50(3):413-5. [Medline].

  10. Aleric I, Krpan M, Peros K. Bronchial adenoid cystic carcinoma presenting as unilateral hyperlucent lung (Swyer-James-McLeod syndrome). Wien Klin Wochenschr. 2007;119(23-24):711. [Medline].

  11. Ghossain MA, Achkar A, Buy JN. Swyer-James syndrome documented by spiral CT angiography and high resolution inspiratory and expiratory CT: an accurate single modality exploration. J Comput Assist Tomogr. Jul-Aug 1997;21(4):616-8. [Medline].

  12. Dornia C, Pfeifer M, Hamer OW. [MacLeod-Swyer-James syndrome--incidental CT finding in a 21-year-old patient]. Rofo. Apr 2008;180(4):351-3. [Medline].

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Further Reading

Pulmonary rehabilitation: joint ACCP/AACVPR evidence-based clinical practice guidelines.
American Association of Cardiovascular and Pulmonary Rehabilitation - Medical Specialty Society
American College of Chest Physicians - Medical Specialty Society.  1997 (revised 2007 May).  39 pages.  NGC:005669

Chronic obstructive pulmonary disease. National clinical guideline on management of chronic obstructive pulmonary disease in adults in primary and secondary care.
National Collaborating Centre for Chronic Conditions - National Government Agency [Non-U.S.].  2004 Feb.  232 pages.  NGC:003545
 
Global strategy for the diagnosis, management, and prevention of chronic obstructive pulmonary disease.
Global Initiative for Chronic Obstructive Lung Disease - Disease Specific Society
National Heart, Lung, and Blood Institute (U.S.) - Federal Government Agency [U.S.]
World Health Organization - International Agency.  2006 (revised 2007).  109 pages.  NGC:006275

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Keywords

Swyer-James syndrome, MacLeod syndrome, hyperlucent lung, hyperlucent thorax, unilateral hyperlucent lung, hypogenetic lung, SJS, pulmonary hyperlucency, pulmonary emphysema

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Contributor Information and Disclosures

Author

Beverly P Wood, MD, MS, PhD, Professor, Departments of Radiology and Pediatrics, Division of Medical Education, Keck School of Medicine, University of Southern California
Beverly P Wood, MD, MS, PhD is a member of the following medical societies: American Academy of Pediatrics, American Association for Women Radiologists, American College of Radiology, American Institute of Ultrasound in Medicine, American Medical Association, American Roentgen Ray Society, Association of University Radiologists, Radiological Society of North America, and Society for Pediatric Radiology
Disclosure: Nothing to disclose.

Medical Editor

Lori Lee Barr, MD, FACR, FAIUM, Clinical Associate Professor of Radiology, University of Texas Health Science Center in San Antonio; Clinical Assistant Professor of Radiology, University of Texas Medical Branch at Galveston; Member, Board of Directors, Austin Radiological Association; Consulting Staff, Seton Health Network, Columbia/St David's Healthcare System, Healthsouth Rehabilitation Hospital of Austin, Georgetown Hospital, St Mark's Medical Center, Cedar Park Regional Medical Center
Lori Lee Barr, MD, FACR, FAIUM is a member of the following medical societies: American Association for Women Radiologists, American College of Radiology, American Institute of Ultrasound in Medicine, American Roentgen Ray Society, American Society of Pediatric Neuroradiology, Association of University Radiologists, Radiological Society of North America, Society for Pediatric Radiology, Society of Radiologists in Ultrasound, Southern Medical Association, Texas Radiological Society, and Undersea and Hyperbaric Medical Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.

Managing Editor

Eric J Stern, MD, Professor of Radiology, Adjunct Professor of Medicine, Adjunct Professor of Medical Education and Biomedical Informatics, University of Washington School of Medicine; Director of Thoracic Imaging, Harborview Medical Center; Associate Medical Staff, Seattle Cancer Care Alliance
Eric J Stern, MD is a member of the following medical societies: American Roentgen Ray Society, Association of University Radiologists, European Society of Radiology, Radiological Society of North America, and Society of Thoracic Radiology
Disclosure: Nothing to disclose.

CME Editor

Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.

Chief Editor

Eugene C Lin, MD, Clinical Assistant Professor of Radiology, University of Washington Medical School
Eugene C Lin, MD is a member of the following medical societies: American College of Nuclear Medicine, American College of Radiology, Radiological Society of North America, and Society of Nuclear Medicine
Disclosure: Nothing to disclose.

 
 
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