Adult Diagnosis of Swyer-James-MacLeod Syndrome: A Case Report

Carlos Capela; Paulo Gouveia; Marco Sousa; Maria J Regadas

Disclosures

J Med Case Reports. 2011;5(1) 

In This Article

Abstract and Introduction

Abstract

Introduction: Swyer-James-MacLeod syndrome or unilateral hyperlucent lung syndrome is a rare entity associated with postinfectious bronchiolitis obliterans occurring in childhood. It is characterized by hypoplasia and/or agenesis of the pulmonary arteries resulting in pulmonary parenchyma hypoperfusion.
Case presentation: Here we report the case of a 53-year-old Caucasian woman with Swyer-James-MacLeod syndrome found in the differential diagnosis workup for a new onset of heart failure, secondary to pulmonary arterial hypertension complicated by a patent ductus arteriosus.
Conclusion: Typically, this disorder is diagnosed in childhood after evaluation for recurrent respiratory infections, but sometimes an indolent course means diagnosis is not made until adulthood.

Introduction

Swyer-James-MacLeod Syndrome (SJMS) or unilateral hyperlucent lung syndrome is a rare entity associated with postinfectious bronchiolitis obliterans occurring in childhood.[1] It is characterized by hypoplasia and/or agenesis of the pulmonary arteries resulting in pulmonary parenchyma hypoperfusion, showing a characteristic radiological pattern, such as translucent or hyperlucent unilateral lung.[2] Typically, this disorder is diagnosed in childhood after an evaluation for recurrent respiratory infections but sometimes patients who have little or no sequelae bronchiectasis have minor symptoms or are asymptomatic and may, therefore, miss their diagnosis until adulthood.[3] Here we presented a 53-year-old woman with SJMS found in the differential diagnosis workup for a new onset of heart failure.

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