Swyer-James-MacLeod Syndrome

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.

No genetic inheritance.

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.