J Korean Soc Neonatol.  2011 Nov;18(2):404-408. 10.5385/jksn.2011.18.2.404.

A Rare Case of Scimitar Syndrome Accompanied with an Imperforate Anus in a Newborn

• Chun P
• Lee HD
• Kim YD

• KMID: 2099223
• DOI: http://doi.org/10.5385/jksn.2011.18.2.404

Abstract

Scimitar syndrome (SS) is a rare congenital anomaly characterized by hypoplasia of the right lung and partially anomalous pulmonary venous drainage to the inferior vena cava. The term scimitar derives from the shadow created by the anomalous pulmonary vein on chest X-ray that closely resembles that of a curved Turkish sword. It rarely presents as an isolated abnormality. Various cardiac and non-cardiac anomalies have been association with SS, such as right lung hypoplasia, dextroposition of the heart, hypoplasia of the right pulmonary artery, systemic arterial blood supply to the right lower lung from the infra-diaphragmatic aorta, and a secundum type of atrial septal defect. However, an imperforate anus has not been reported previously in association with SS. We describe the first case of infantile scimitar syndrome accompanied with an imperforate anus in a newborn who presented with tachypnea and right pulmonary atelectasis.

Keyword

Scimitar syndrome; Imperforate anus; Pulmonary atelectasis

MeSH Terms

Anus, Imperforate
Aorta
Drainage
Heart
Heart Septal Defects, Atrial
Humans
Infant, Newborn
Lung
Pulmonary Artery
Pulmonary Atelectasis
Pulmonary Veins
Scimitar Syndrome
Tachypnea
Thorax
Vena Cava, Inferior