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

Chun,  Peter; Lee,  Hyoung Doo; Kim,  Young Don

doi:2011.18.2.404

Neonatal Med > Volume 18(2); 2011 > Article

Case Report

Journal of the Korean Society of Neonatology 2011;18(2):404-408.
DOI: https://doi.org/10.5385/jksn.2011.18.2.404 Published online November 25, 2011.

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

Peter Chun, Hyoung Doo Lee, Young Don Kim

¹Department of Pediatrics, Pusan National University School of Medicine, Busan, Korea.
²Department of Pediatrics, Jeju National University School of Medicine, Jeju, Korea. cardios@hanmail.net

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.

Key Words: Scimitar syndrome; Imperforate anus; Pulmonary atelectasis