A Neonate with Scimitar Syndrome, Absent Right Pulmonary Artery, Imperforated Anus, and Coarctation of the Aorta

Abstract

Background: Scimitar syndrome is a rare and complex congenital defect that often involves hypoplasia of the right lung, partial anomalous pulmonary venous connection of the right lung draining into the inferior vena cava, and a systemic collateral artery from the descending aorta supplying the right lower lobe (lobar sequestration). Our case was a male newborn with scimitar syndrome associated with an absent right pulmonary artery, imperforated anus, and coarctation of the aorta. Hemodinamically, the patient was asymptomatic and diagnosed accidentally. This combination has not been reported before. The pathological process of the scimitar syndrome is unclear. However, it appears to originate from a basic developmental disorder of the complete pulmonary bud early in embryogenesis. This rare syndrome with different presentations can occur alone or in combination with cardiac and /or non-cardiac anomalies.