Ventilation-Perfusion Scintigraphic Correlation With Multimodality Imaging in a Proven Case of Swyer-James (Macleod's) Syndrome

Abstract

The scintigraphic findings in a rare case of a unilateral matched ventilation perfusion defect, Swyer-James (Macleod's) syndrome, are presented. A 40-year-old man underwent ventilation/perfusion imaging for acute onset of dyspnea. The scan showed markedly diminished ventilation, vascular flow, and perfusion unilaterally on the left hemithorax. Chest radiography showed hyperlucency of the left lung. Pulmonary angiography showed left-sided hypovascularity, and the CT scan of the chest showed apical bullae and peribronchial thickening of the left lung. Ventilation/perfusion imaging and other radiologic assessment, along with the patient's medical history, confirmed the diagnosis of the Swyer-James syndrome. This condition should be considered as a differential diagnosis in a patient with unilaterally matched ventilation/perfusion deficits without an obvious etiology.

Adult diagnosis of Swyer-James-MacLeod syndrome: a case report

“…Finally, another characteristic described is that the pathological perfusion changes in the diseased lung sections and the occasionally bizarre hyperinflation ultimately result in the compression of healthy lung areas resulting in atelectasis [6]. For this reason, the diagnosis of this syndrome is better established with HRCT on inspiration and expiration complemented with an angio-CT. A ventilation-perfusion lung scanning [7] could also be performed but false-positives could appear in the presence of any disorder involving distal airway obstruction (such as, bronchiolitis obliterans, asthma or congenital lobar emphysema). SJMS treatment includes the early control of lung infections as well as influenza and pneumococcal vaccinations [2,5].…”

Classical Imaging Triad in a Very Young Child with Swyer-James Syndrome

“…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 SJS 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 [4][5][6][7]. Any disorder involving distal airway obstruction (bronchiolitis obliterans, foreign body inhalation, congenital lobar emphysema) is a differential diagnosis for SJS.…”

Swyer-James Syndrome with Peculiar Course and Ipsilateral Pulmonary Vein Defect

“…The ventilation and perfusion scans of SJS show the characteristic pattern of a matched V/Q defect and diffusely delayed washout in the affected lung (24). Magnetic resonance angiography or digital subtraction angiography, necessary, can be used to confirm hypoplasia of pulmonary artery.…”