Swyer-James-MacLeod syndrome with ipsilateral herniation of hyperinflated hyperlucent lung

Area of Science:

• Pulmonology
• Radiology
• Medical Imaging

Background:

• Swyer-James-MacLeod syndrome (SJMS) is a rare congenital or acquired condition.
• It is characterized by unilateral lung hyperlucency, reduced lung size on the affected side, and compensatory hyperinflation of the contralateral lung.
• The syndrome typically results from post-infectious obliterative bronchiolitis.

Observation:

• This report describes a rare case of SJMS in an adult female.
• The patient presented with exertional breathlessness.
• Imaging revealed a hypoplastic left pulmonary artery, leading to a hyperlucent, hyperinflated, and herniated left lung.

Findings:

• The key finding is the rare presentation of a hyperinflated, rather than hypoplastic, ipsilateral lung in SJMS.
• The presence of a hypoplastic pulmonary artery was identified as the underlying cause in this specific case.
• Lung herniation was also noted as an unusual associated finding.

Implications:

• This case expands the understanding of SJMS, highlighting that ipsilateral lung hyperinflation can occur.
• It underscores the importance of considering vascular anomalies, such as pulmonary artery hypoplasia, in the etiology of SJMS.
• The findings suggest that imaging findings in SJMS can be more varied than previously thought, necessitating a comprehensive diagnostic approach.