Scimitar syndrome – a case report

Abstract

Objectives The aim of this article is to describe a clinical case of Scimitar syndrome, an unusual cause of respiratory distress in the neonatal period. This syndrome is a rare form of partial or total anomalous right lung venous drainage to the inferior caval vein, usually associated with right pulmonary hypoplasia. Case presentation The authors report a case of a 36-week-old newborn admitted to the neonatal intensive care unit with worsening respiratory distress. Chest radiography revealed a characteristic right pulmonary hypoplasia and a tubular structure paralleling the right heart border in the shape of a Turkish sword (“scimitar”). Chest computed tomography angiography confirmed the diagnosis. Conclusions Age of presentation and the severe malformations found, contributed to the patient’s poor outcome in this case.