Swyer-James Syndrome Imaging

Updated: Nov 18, 2015
  • Author: Beverly P Wood, MD, MSEd, PhD; Chief Editor: Eugene C Lin, MD  more...
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Overview

Overview

Swyer-James syndrome (SJS) is a manifestation of postinfectious obliterative bronchiolitis. In SJS, the involved lung or portion of the lung does not grow normally and is slightly smaller than the opposite lung. The characteristic radiographic appearance is that of pulmonary hyperlucency, caused by overdistention of the alveoli in conjunction with diminished arterial flow. [1, 2, 3] (See the images below.)

Anteroposterior chest radiograph of a 12-month-old Anteroposterior chest radiograph of a 12-month-old patient indicates diffuse pneumonia that is clearing on the right.
Radiograph of the same infant shown in the previou Radiograph of the same infant shown in the previous image, now 20 months of age. The pneumonia has cleared. The left lung is hyperlucent and is overexpanded. The right lung is small. The patient was asymptomatic at this time.

Swyer-James syndrome refers to an inability of the lungs to grow after a severe obstructive bronchiolitis. On CT scan, the affected lung shows small size and decreased vascularity, hyperexpansion and hyperlucency, and, rarely, bronchiectasis. For patients with Swyer-James syndrome, chest CT scanning with thin collimation sections on inspiration and expiration is the preferred examination. [4, 5] The appearance of the lungs on forced expiration is important in the assessment of SJS with CT scanning; therefore, the patient's cooperation is essential. The patient should be placed in the prone position to help identify the typical mosaic pattern of the syndrome.Small regions of hyperlucency representing air trapping are reported in some patients. Adenovirus infection is considered the most usual epidemiology. Patients respond well to management with bronchodilators, even though this is not primarily a bronchial abnormality.

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Radiography

Radiographically, the imaging findings of SJS appear a few months to a few years after the causative infection. The typical appearance of Swyer-James syndrome is that of a small, hyperlucent lung, with overexpansion of the contralateral lung. A diffuse pattern of scarring or irregular vessels may be present.

A comparison of progressive radiographs shows failure of growth in the involved lung. Fluoroscopy shows little change in volume in the involved lung with respiration. (See images below.)

Anteroposterior chest radiograph of a 12-month-old Anteroposterior chest radiograph of a 12-month-old patient indicates diffuse pneumonia that is clearing on the right.
Radiograph of the same infant shown in the previou Radiograph of the same infant shown in the previous image, now 20 months of age. The pneumonia has cleared. The left lung is hyperlucent and is overexpanded. The right lung is small. The patient was asymptomatic at this time.
X-ray of a 4-month-old child shows pneumonia centr X-ray of a 4-month-old child shows pneumonia centrally throughout the right lung. Pneumonia was caused by a severe respiratory syncytial virus infection.
Same child shown in the previous image, now 8 year Same child shown in the previous image, now 8 years of age. The patient was experiencing severe wheezing and episodes of hypoxemia. Note the small right lung and overexpansion of the left lung.
A febrile, ill, 3-month-old infant with rales and A febrile, ill, 3-month-old infant with rales and rhonchi, which are more severe on the left than the right.
The same patient shown in the previous image, now The same patient shown in the previous image, now 12 years of age. Frontal radiograph shows the left lung to be somewhat small and hyperlucent. The organism that caused the original pneumonia was cytomegalovirus.

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.

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Computed Tomography

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. [6, 4, 5]

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

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Magnetic Resonance Imaging

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

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Nuclear Imaging

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.

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

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Angiography

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.

However, acquired hypoplastic lung cannot be differentiated on angiography from congenital hypoplasia of the lung. Swyer-James syndrome is also similar in appearance to lobar emphysema and hypoplastic pulmonary artery. [7]

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