Swyer-James-Macleod syndrome: report of three cases

Abstract

Introduction: Swyer–James–Macleod Syndrome (SJMS) is a rare disorder (frequency: 0,01%), characterized by unilateral hyperlucent lungs. It arises from impairment of vascular development in an area affected by obliterans bronchiolitis in childhood. As a result hypoplastic vascular regions and emphysematous areas occur (most commonly on the left side) resulting in the characteristic radiological feature of an hyperlucent lung. The long-term clinical course has not been clearly described yet. Objectives: Review of the cases followed at CHVNGE in 2016. Results: Three cases were identified (table 1). Conclusion: All patients presented with respiratory symptoms. The hyperlucent zones were only on the left lung, with bronchiectasis and obstructive ventilatory defect in the 3 cases. Two patients reported respiratory infections in childhood. All these features have been described in the literature. Although there are no recommendations regarding the follow-up of this entity, since some severe complications have been reported (eg pulmonary hypertension) monitoring may be necessary.