Clinical, physiologic, and roentgenographic changes after pneumonectomy in a boy with Macleod/Swyer‐James syndrome and bronchiectasis

Abstract

Macleod/Swyer-James syndrome is an uncommon and complex disease characterized by roentgenographic hyperlucency of one lung or lobe due to loss of the pulmonary vascular structure and to alveolar overdistension. This syndrome seems to be an acquired disease that follows viral bronchiolitis and pneumonitis in childhood. It must be differentiated from many other causes of unilateral lung "transradiancy" on the chest roentgenogram, such as those related to congenital bronchial and/or vascular abnormalities. We here describe an 11-year-old patient with Macleod/Swyer-James syndrome and bronchiectasis resulting in severe recurrent bronchopulmonary infections. Despite the severe impairment of pulmonary function, the patient underwent resection of the right lung with progressive improvement of clinical and physiologic parameters.

Swyer-James Syndrome Diagnosed in a Trauma Patient

“…In addition, CT scan has been shown to have a higher sensitivity for detection of hypoplastic lung lobes, bronchiectasis, and pulmonary bronchus size when compared to standard end expiratory frontal chest X-ray studies (14). When diagnosed, treatment of SJS is typically conservative and supportive, including close follow-up and management of often recurrent pulmonary infections, and rarely with lung resections for recalcitrant disease (6,7,15).…”

“…B. Fortune ratory syncytial virus, resulting in bronchiolitis and, ultimately, destruction of terminal bronchial branches, lung hypoperfusion, and developmental delay of alveolar buds (5)(6)(7). This phenomenon is rare, with a prevalence of 0.01% of chest X-ray studies showing signs of the syndrome (6).…”

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 vascular ring in a child: Echocardiogrpahic diagnosis

“…[9] Prevention and management of recurrent respiratory tract infections is the main treatment for SJS. [10] If infections of the ectatic bronchi cannot be controlled, advanced treatment to remove a diseased and chronically infected lung to allow normal development of lung parenchyma is essential for improvement of quality of life and life expectancy. VR with ARSCA, a sling effect on trachea and esophagus with variable degree of airway obstruction, is usually a cause of respiratory tract infection.…”