Swyer-James-Macleod Syndrome

At a glance

A rare acquired syndrome characterized by characterized by hyperlucency of one lung, lobe or part of a lobe. It is probably a manifestation of postinfectious obliterative bronchiolitis leading to a smaller, radiologically hyperlucent, affected lung.

Synonyms

Hyperlucent Lung Syndrome; MacLeod Syndrome.

Incidence

Rare, occurs in approximately 4% of all patients with bronchiolitis obliterans.

Genetic inheritance

No genetic inheritance.

Pathophysiology

Lung grows by progressive alveolarization, generally before the age of 8 years. Following an infection, a reduced pulmonary perfusion 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, Bordetella pertussis, Pseudomonas aeruginosa, and Streptococcus pneumoniae.

Diagnosis

Apparent 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.

Clinical aspects

Involves only respiratory function with hyperlucency, air trapping upon expiration, wheezing, and abnormal time attenuation curves during inspiration and forced expiration.

Precautions before anesthesia

Evaluate respiratory function (clinical, pulmonary function test with arterial blood gas analysis, CT scan). Chronic respiratory infections are common and must be optimized prior to surgery.

Anesthetic considerations

The use of positive pressure ventilation support must be done carefully and the potential for sudden pneumothorax must be considered in the presence of hemodynamic instability. Perioperative physiotherapy is needed to avoid pulmonary superinfection.

Pharmacological implications

Probably best to avoid nitrous oxide because it can increase the risk of pneumothorax.

References