A rare acquired syndrome characterized by a probable
manifestation of postinfectious obliterative bronchiolitis leading to a
smaller, radiologically hyperlucent, affected lung.
Hyperlucent Lung Syndrome; MacLeod Syndrome.
Lung grows by progressive alveolarization,
generally before the age of 8 years. Following an infectious syndrome, a
reduced pulmonary perfusion that leads to an arrest of progressive growth
and alveolarization of the lung. Swyer-James-Macleod Syndrome generally
follows a severe bronchiolitis but has also been described following
infection by Mycoplasma pneumoniae and Streptococcus pneumoniae.
Evocated a few months or years after an infection in a
child with respiratory symptoms and eventually an asymmetric chest, or on a
systematic chest radiograph with a hyperlucent lateralized aspect.
Involves only respiratory function with
hyperlucency, air trapping upon expiration, wheezing, and abnormal
time-attenuation curves during inspiration and forced expiration.
Evaluate respiratory function
(clinical, pulmonary function test with arterial blood gas analysis, CT scan).
The use of positive pressure ventilation support
must be done carefully and the potential for sudden pneumothorax must be kept in mind in
pesence of hemodynamic instability. Perioperative physiotherapy is
needed to avoid pulmonary superinfection.
Probably best to avoid nitrous oxide
because it can increase the risk of pneumothorax.
Braunschweig M, Gal I: Swyer-James syndrome. JBR-BTR 84(2):57, 2001.
Fregonese L, Girosi D, Battistini E, et al: Physiologic and
roentgenographic changes after pneumonectomy in a boy with
Macleod/ Swyer-James syndrome and bronchiectasis. Pediatr Pulmonol 34(5):412, 2002.